Information on EC 6.3.4.5 - Argininosuccinate synthase:
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EC NUMBERCOMMENTARY
6.3.4.5-

RECOMMENDED NAMEGeneOntology No.
Argininosuccinate synthaseGO:0004055

REACTIONREACTION DIAGRAMCOMMENTARYORGANISM UNIPROT ACCESSION NO.LITERATURE
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		----
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		mechanismBos taurus-1574
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		rapid-equilibrium random mechanismSaccharomyces cerevisiae-1579
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		ordered addition of MgATP2-, citrulline, and aspartate, followed by ordered release of argininosuccinate, magnesiumdiphosphate and AMPBos taurus-1587
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		essential Arg in the active site participates in the binding of ATP and diphosphate. The binding of ATP and diphosphate at the same site is mutually exclusiveBos taurus-1589
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		-Escherichia coli-649104
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		-Homo sapiens-650761, 652976
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		-Mus musculus-650761, 653046, 653186
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		-Canis lupus familiaris-650761
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		-Rattus norvegicus-651195, 653186, 653344
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		detailed catalytic mechanismEscherichia coli-652231
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		catalytic mechanismThermus thermophilus-652242
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		detailed catalytic mechanism, stereochemistryThermus thermophilusP59846652404
ATP + L-citrulline + L-aspartate = AMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		it is found that argininosuccinate synthetase is the major lipid A-interacting protein in liver. Argininosuccinate synthetase also inhibits the biological activities of natural lipid A and rough-type lipopolysaccharide and but not smooth-type lipopolysaccharide. Argininosuccinate synthetase enzyme activity is inhibited by lipid A and rough-type lipopolysaccharide and smooth-type lipopolysaccharide.Mus musculus-675096

REACTION TYPEORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
amination----

PATHWAYKEGG LinkMetaCyc Link
arginine biosynthesis I-ARGSYN-PWY
arginine biosynthesis II (acetyl cycle)-ARGSYNBSUB-PWY
arginine biosynthesis III-PWY-5154
arginine biosynthesis IV-ARGININE-SYN4-PWY
canavanine biosynthesis-PWY-5
citrulline-nitric oxide cycle-PWY-4983
urea cycle-PWY-4984

SYSTEMATIC NAMEIUBMB Comments
L-Citrulline:L-aspartate ligase (AMP-forming)-

SYNONYMSORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
ARGGLeishmania infantumA4I066-703594
Arginine succinate synthetase----
argininosuccinate synthaseBos taurus--671250
Argininosuccinate synthetase----
Argininosuccinate synthetaseEscherichia coli--649104, 652231
Argininosuccinate synthetaseHomo sapiens--650761, 652976, 672445, 674048, 674383, 690234, 690768, 692659, 703248, 703251, 703969, 704078, 704509, 705506
Argininosuccinate synthetaseMus musculus--650761, 653046, 653186, 675096, 704509
Argininosuccinate synthetaseCanis lupus familiaris--650761
Argininosuccinate synthetaseRattus norvegicus--651195, 653186, 653344, 673486
Argininosuccinate synthetaseThermus thermophilus--652242, 652404
Argininosuccinate synthetasePorphyra yezoensisQ0KKQ0-675606
Argininosuccinate synthetaseAgaricus bisporusQ2Z0F6-676120
Argininosuccinate synthetaseDaucus carota, Glomus intraradices--676636
Argininosuccinate synthetaseLeishmania infantumA4I066-703594
Argininosuccinic acid synthetase----
arininosuccinate synthetaseHomo sapiens--704070
ASEscherichia coli--649104, 652231
ASMus musculus--650761, 653046, 653186
ASCanis lupus familiaris, Homo sapiens--650761
ASRattus norvegicus--653186
ASS----
ASSThermus thermophilus--652242, 652404
ASSHomo sapiens--652976, 672445, 690234, 690768, 692659, 704509, 705506
ASSRattus norvegicus--653344, 673486
ASSAgaricus bisporusQ2Z0F6-676120
ASSMus musculusP16460-704509
ASS1Homo sapiens--703969, 704070, 704078, 715624
Citrulline--aspartate ligase----
Citrulline-aspartate ligase----
Citrulline-aspartate ligaseHomo sapiensP00966-690234
EASEscherichia coli--649104, 652231
Elastin-binding protein----
PyARG1Porphyra yezoensisQ0KKQ0-675606
Synthetase, argininosuccinate----
tAsSThermus thermophilus--652242, 652404

CAS REGISTRY NUMBERCOMMENTARY
9023-58-9-

ORGANISMCOMMENTARYLITERATURESEQUENCE CODESEQUENCE DB SOURCE
Agaricus bisporus-676120Q2Z0F6SwissProtManually annotated by BRENDA team
Bos taurus-1574, 1577, 1585, 1587, 1588, 1589, 662205, 662377, 671250, 690727, 690765--Manually annotated by BRENDA team
Canis lupus familiaris-650761--Manually annotated by BRENDA team
Daucus carota-676636--Manually annotated by BRENDA team
Escherichia coli-649104, 652231--Manually annotated by BRENDA team
Felis catus-1590--Manually annotated by BRENDA team
Glomus intraradices-676636--Manually annotated by BRENDA team
Homo sapiens-1578, 1581, 1583, 1586, 1595, 650761, 661285, 662254, 672445, 674048, 674383, 690768, 692659, 703251, 704070, 704078, 704509, 705506--Manually annotated by BRENDA team
Homo sapiens-693030, 703248, 703969, 715188, 715624P00966UniProtManually annotated by BRENDA team
Homo sapiensadult-type citrullinemia with neuro-psychiatric symptoms of late onset, 24-28 years old, caused by ASS abnormalities1584--Manually annotated by BRENDA team
Homo sapiensASS1690234P00966UniProtManually annotated by BRENDA team
Homo sapienscitrullinemia is an autosomal recessive disease caused by a deficiency of argininosuccinate synthetase; wild-type and mutant enzyme; wild-type and mutant enzymes1598--Manually annotated by BRENDA team
Homo sapienspatients with Alzheimer disease and nondemented, age-matched controls652976--Manually annotated by BRENDA team
Leishmania infantumantimonial-sensitive and antimonial-resistant strains703594A4I066UniProtManually annotated by BRENDA team
Mus musculus-1593, 650761, 653186, 675096, 715188--Manually annotated by BRENDA team
Mus musculus-704509P16460UniProtManually annotated by BRENDA team
Mus musculushuman tumor necrosis factor-alpha transgenic mice, 4 and 8 weeks old, and age-matched CBA x C57B1/6 wild-type controls653046--Manually annotated by BRENDA team
Mus musculusmale C3H/He, C57BL/6, and BALB/c mice692607--Manually annotated by BRENDA team
Pisum sativum-1573--Manually annotated by BRENDA team
Porphyra yezoensis-675606Q0KKQ0SwissProtManually annotated by BRENDA team
Rattus norvegicus-1575, 1576, 1580, 1582, 1584, 1586, 1591, 1592, 1594, 1597, 673486--Manually annotated by BRENDA team
Rattus norvegicusmale Wistar rats651195--Manually annotated by BRENDA team
Rattus norvegicusSprague-Dawley rats653344--Manually annotated by BRENDA team
Rattus norvegicusWistar rats, 2 days old653186--Manually annotated by BRENDA team
Saccharomyces cerevisiaestrain 1278b, mutant MG4091579--Manually annotated by BRENDA team
Saccharomyces cerevisiae 1278bstrain 1278b, mutant MG4091579--Manually annotated by BRENDA team
Sus scrofa-1596--Manually annotated by BRENDA team
Thermus thermophilusstrain HB8652242--Manually annotated by BRENDA team
Thermus thermophilusstrain HB8652404P59846UniprotManually annotated by BRENDA team

GENERAL INFORMATIONORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
malfunctionHomo sapiens-mutation results in citrullinemia type I703248
malfunctionHomo sapiens-malfunction results in citrullinemia type 1 argininosuccinate argininosuccinate synthetase deficiency703251
malfunctionHomo sapiens-deficiency of the enzyme may result in citrullinemia type I703969
malfunctionHomo sapiens-epigenetic silencing triggers apoptosis in arginine auxotroph tumours704078
malfunctionHomo sapiens-enzyme deficency results in congenital urea cycle disorders705506
physiological functionHomo sapiens-involved in urea cycle703248, 703969, 705506
physiological functionHomo sapiens-rate-limiting enzyme in the biosynthesis of arginine704070
physiological functionHomo sapiens-rate-limiting enzyme in urea and arginine(NO)-citrulline cycles704078
physiological functionHomo sapiens-part of citrulline-NO cycle, limiting step in NO synthesis704509
physiological functionMus musculusP16460part of citrulline-NO cycle, limiting step in NO synthesis704509
physiological functionMus musculus-recombinant argininosuccinate synthase reduces lipolysaccharide cytotoxicity, TNF-alpha production, and increases cell viability in cultured mouse macrophages, even when added one h following lipopolysaccharide challenge. Intraperitoneal injection of recombinant argininosuccinate synthase at 5 mg/kg after treatment with a high dose of lipopolysaccharide remarkably increases survival of rodents, with a concomitant decrease of sepsis markers TNF-alpha, C-reactive protein, and lactate dehydrogenase levels in blood715188
physiological functionHomo sapiens-exogenous expression of either nitric oxide synthase NOS3 or argininosuccinate synthetaseASS1 increases NO production and decreases monocyte adhesion stimulated by tumor necrosis factor-alpha. The latter effect is reduced when human umbilical vein endothelial cells are co-treated with small interfering RNAs for ASS1 or NOS3. SiRNAs of NOS3 and ASS1 attenuate the increase of NO production in human aortic endothelial cells stimulated by laminar shear stress for 24 h715624

SUBSTRATEPRODUCT                      REACTION DIAGRAMORGANISM UNIPROT ACCESSION NO. COMMENTARY/
Substrate		 LITERATURE/
Substrate		 COMMENTARY/
Product		 LITERATURE/
Product		 Reversibility
r=reversible
ir=irreversible
?=not specified
ATP + citrulline + aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Homo sapiens--1583, 1586, 1598---
ATP + citrulline + aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Rattus norvegicus--1575, 1576---
ATP + citrulline + aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Bos taurus--1577---
ATP + citrulline + aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Mus musculusP16460assay at pH 7.8, 37°C, reaction stopped by addition of molybdate buffer704509--?
ATP + L-citrulline + 3-nitro-2-aminopropanoate?
show the reaction diagram		Bos taurus-60% of the maximal activity with Asp1587---
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Mus musculus--1593-1593-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Homo sapiens--1578-1578-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Homo sapiens--1581-1581-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Homo sapiens--1583-1583-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Homo sapiens--1584-1584-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Homo sapiens--1586-1586-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Homo sapiens--1595-1595-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Homo sapiens--1598-1598-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus--1575-1575-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus--1580-1580-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus--1582-1582-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus--1584-1584-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus--1586-1586-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus--1591-1591-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus--1592-1592-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus--1594-1594-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus--1597-1597-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Sus scrofa--1596-1596-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Bos taurus--1577-1577-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Bos taurus--1585-1585-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Bos taurus--1587-1587-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Bos taurus--1588-1588-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Bos taurus--1589-1589-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Pisum sativum--1573-1573-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Felis catus--1590-1590-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus-r1576-1576-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Saccharomyces cerevisiae-r1579-1579-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Bos taurus-r, D-Asp is inactive as substrate1574-1574-
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Bos taurus-argininosuccinate synthase catalyzes the rate-limiting step in the recycling of citrulline to arginine, which in endothelial cells, is tighly coupled to the production of nitric oxide. The upstream open reading frame found in the extended, overlapping 5'-untranslated region of argininosuccinate synthase mRNA species suppresses endothelial argininosuccinate expression providing a novel mechanism for regulating endothelial NO production by limiting the availability of arginine662377--?
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Bos taurus-argininosuccinate synthase expression is required to maintain nitric oxide production and cell viability in aortic endothelial cells662205--?
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Homo sapiens-IL-1beta stimulates argininosuccinate synthetase gene expression through NF-kappaB in Caco-2 cells661285--?
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Homo sapiens-the substrate for NO synthesis, L-arginine can be regenerated from the L-citrulline the coproduct of nitric-oxide synthase. This requires the sequential action of two enzymes, argininosuccinate synthetase and argininosuccinate lyase662254--?
ATP + L-citrulline + L-Asp?
show the reaction diagram		Rattus norvegicus-mechanism to recycle citrulline, formed in the nervous system via nitric oxide synthetase, EC 1.14.13.39, activity, back to the nitric oxide precursor, L-Arg1594---
ATP + L-citrulline + L-Asp?
show the reaction diagram		Rattus norvegicus-third reaction of urea cycle1591---
ATP + L-citrulline + L-Asp?
show the reaction diagram		Saccharomyces cerevisiae-anabolic function in biosynthesis of Arg and catabolic function as the first enzyme of citrulline utilization as nitrogen source1579---
ATP + L-citrulline + L-Asp?
show the reaction diagram		Homo sapiens-urea-cycle enzyme1598---
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Mus musculus--675096--?
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Homo sapiens--652976-652976?
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus--651195-651195?
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus--653344-653344?
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Glomus intraradices--676636--?
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Escherichia coli-ATP induces conformational changes in the nucleotide binding domain, each monomer of tetrameric EAS consists of a nucleotide binding domain and a novel catalytic/multimerization domain, detailed catalytic mechanism via adenylated citrulline intermediate, active site structure652231-652231r
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Thermus thermophilusP59846ATP-dependent condensation of citrulline with aspartate, detailed mechanism via citrullyl-AMP intermediate, stereochemistry of catalysis, no conformational change upon binding of ATP, active site structure652404-652404r
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Thermus thermophilus-ATP-dependent condensation of citrulline with aspartate, no conformational change upon binding of substrates, mechanism via citrullyl-AMP intermediate, active site structure652242-652242r
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Escherichia coli-ATP-dependent ligation of citrulline and aspartate649104-649104r
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Mus musculus, Homo sapiens, Canis lupus familiaris-catalyzes the coupling reaction between citrulline and arginine to form argininosuccinic acid650761-650761?
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus-mediates transformation of L-citrulline to L-arginine in the ureum cycle in hepatocytes, first step in L-citrulline recycling pathway651195-651195?
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Escherichia coli-penultimate step in the biosnthesis of arginine, rate-limiting enzyme of both the urea and arginine-citrulline cycles652231-652231?
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Thermus thermophilus-second step of urea cycle652242-652242?
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Thermus thermophilusP59846seventh step of the arginine biosynthesis, second step of the urea cycle652404-652404?
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Mus musculus, Homo sapiens, Canis lupus familiaris-the rate-limiting enzyme for converting citrulline to arginine650761-650761?
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Mus musculus--692607--?
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Homo sapiens--690768, 692659--?
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Homo sapiensP00966-693030--?
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Bos taurus--690727, 690765--?
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Bos taurus-argininosuccinate synthase is essential for endothelial nitric oxide production, vascular NO production is regulated by dynamic argininosuccinate synthase phosphorylation690765--?
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Homo sapiensP00966citrulline-aspartate ligase is the rate-limiting enzyme in the synthesis of the amino acid arginine. Type I citrullinaemia is a disorder caused by mutations in the ASS gene leading to reduced or abolished activity of the ASS enzyme690234--?
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Homo sapiensP00966rate-limiting enzyme in nitric oxide synthesis, negative feedback mechanism, overview693030--?
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Mus musculus-rate-limiting enzyme of the urea cycle692607--?
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Bos taurus-the caveolar-localized endothelial argininosuccinate synthase catalyzes the rate-limiting step of the citrulline-NO cycle, involving not only the regulation of endothelial nitric oxide synthase, but also regulation of cycle, overview690727--?
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Homo sapiens-the key enzyme in the urea cycle participates in many metabolic processes including arginine biosynthesis and the citrulline-nitric oxide cycle690768--?
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Homo sapiensP00966substrate binding structures, overview690234--?
ATP + L-citrulline + threo-beta-hydroxy-DL-Asp?
show the reaction diagram		Bos taurus--1574---
ATP + L-citrulline + threo-beta-methyl-L-Asp?
show the reaction diagram		Bos taurus--1574---
dATP + L-citrulline + L-AspdAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus-2.6% as active as ATP1576---
additional information?-Homo sapiens-citrullinemia is an autosomal recessive disease caused by a deficiency of argininosuccinate synthetase1598---
additional information?-Homo sapiens-adult-type citrullinemia with neuro-psychiatric symptoms of late onset, 24-28 years old, caused by argininosuccinate synthetase abnormalities1584---
additional information?-Escherichia coli-urea cycle649104-649104?
additional information?-Mus musculus-involved in L-arginine synthesis from L-citrulline, urea cycle, rate-limiting enzyme for high output NO generation, inducible enzyme, AS synthesis is more strictly regulated at the posttranscriptional than the transcriptional level in vivo653046-653046?
additional information?-Rattus norvegicus-involved in the recycling of L-citrulline to L-arginine653344-653344?
additional information?-Homo sapiens-rate-limiting enzyme in the metabolic pathway leading from L-citrulline to L-arginine, urea cycle652976-652976?
additional information?-Mus musculus, Rattus norvegicus-urea cycle, enzyme of citrulline-arginine recycling, which is important for high output production of NO in activated microglial cells, inducible enzyme653186-653186?
additional information?-Mus musculus-ASS binds to and inactivates lipopolysaccharide and lipid A, overview692607---
additional information?-Homo sapiens-factors like diets, hormones and pro-inflammatory stimuli regulate ASS gene expression primarily at the transcription level690768---
additional information?-Homo sapiensP00966in mammals, the urea-cycle enzymes ASS and argininosuccinatelyase are part of an additional pathway together with the enzyme nitric oxide synthase, forming the arginine-citrulline cycle690234---
additional information?-Homo sapiens-pancreatic cancer cell lines deficient in argininosuccinate synthetase are sensitive to arginine deprivation by arginine deiminase692659---
additional information?-Homo sapiensP00966the enzyme interacts and cooperates with NADPH sensor protein, HSCARG, in downregulation of nitric oxide synthesis, the enzyme interacts with the dimerization region of HSCARG, amino acid residues 153-189, HSCARG regulation of AS activity is crucial for maintaining the intracellular balance between redox state and nitric oxide levels, overview693030---

NATURAL SUBSTRATESNATURAL PRODUCTSREACTION DIAGRAMORGANISM UNIPROT ACCESSION NO.COMMENTARY SUBSTRATELITERATURE
(Substrate)		COMMENTARY PRODUCTLITERATURE
(Product)
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Bos taurus-argininosuccinate synthase catalyzes the rate-limiting step in the recycling of citrulline to arginine, which in endothelial cells, is tighly coupled to the production of nitric oxide. The upstream open reading frame found in the extended, overlapping 5'-untranslated region of argininosuccinate synthase mRNA species suppresses endothelial argininosuccinate expression providing a novel mechanism for regulating endothelial NO production by limiting the availability of arginine662377--
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Bos taurus-argininosuccinate synthase expression is required to maintain nitric oxide production and cell viability in aortic endothelial cells662205--
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Homo sapiens-IL-1beta stimulates argininosuccinate synthetase gene expression through NF-kappaB in Caco-2 cells661285--
ATP + L-citrulline + L-AspAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Homo sapiens-the substrate for NO synthesis, L-arginine can be regenerated from the L-citrulline the coproduct of nitric-oxide synthase. This requires the sequential action of two enzymes, argininosuccinate synthetase and argininosuccinate lyase662254--
ATP + L-citrulline + L-Asp?
show the reaction diagram		Rattus norvegicus-mechanism to recycle citrulline, formed in the nervous system via nitric oxide synthetase, EC 1.14.13.39, activity, back to the nitric oxide precursor, L-Arg1594--
ATP + L-citrulline + L-Asp?
show the reaction diagram		Rattus norvegicus-third reaction of urea cycle1591--
ATP + L-citrulline + L-Asp?
show the reaction diagram		Saccharomyces cerevisiae-anabolic function in biosynthesis of Arg and catabolic function as the first enzyme of citrulline utilization as nitrogen source1579--
ATP + L-citrulline + L-Asp?
show the reaction diagram		Homo sapiens-urea-cycle enzyme1598--
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Rattus norvegicus-mediates transformation of L-citrulline to L-arginine in the ureum cycle in hepatocytes, first step in L-citrulline recycling pathway651195-651195
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Escherichia coli-penultimate step in the biosnthesis of arginine, rate-limiting enzyme of both the urea and arginine-citrulline cycles652231-652231
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Thermus thermophilus-second step of urea cycle652242-652242
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Thermus thermophilusP59846seventh step of the arginine biosynthesis, second step of the urea cycle652404-652404
ATP + L-citrulline + L-aspartateAMP + diphosphate + L-argininosuccinate
show the reaction diagram		Mus musculus, Homo sapiens, Canis lupus familiaris-the rate-limiting enzyme for converting citrulline to arginine650761-650761
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Homo sapiens--692659--
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Bos taurus-argininosuccinate synthase is essential for endothelial nitric oxide production, vascular NO production is regulated by dynamic argininosuccinate synthase phosphorylation690765--
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Homo sapiensP00966citrulline-aspartate ligase is the rate-limiting enzyme in the synthesis of the amino acid arginine. Type I citrullinaemia is a disorder caused by mutations in the ASS gene leading to reduced or abolished activity of the ASS enzyme690234--
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Homo sapiensP00966rate-limiting enzyme in nitric oxide synthesis, negative feedback mechanism, overview693030--
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Mus musculus-rate-limiting enzyme of the urea cycle692607--
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Bos taurus-the caveolar-localized endothelial argininosuccinate synthase catalyzes the rate-limiting step of the citrulline-NO cycle, involving not only the regulation of endothelial nitric oxide synthase, but also regulation of cycle, overview690727--
ATP + L-citrulline + L-aspartateAMP + diphosphate + 2-(Nomega-L-arginino)succinate
show the reaction diagram		Homo sapiens-the key enzyme in the urea cycle participates in many metabolic processes including arginine biosynthesis and the citrulline-nitric oxide cycle690768--
additional information?-Homo sapiens-citrullinemia is an autosomal recessive disease caused by a deficiency of argininosuccinate synthetase1598--
additional information?-Homo sapiens-adult-type citrullinemia with neuro-psychiatric symptoms of late onset, 24-28 years old, caused by argininosuccinate synthetase abnormalities1584--
additional information?-Escherichia coli-urea cycle649104-649104
additional information?-Mus musculus-involved in L-arginine synthesis from L-citrulline, urea cycle, rate-limiting enzyme for high output NO generation, inducible enzyme, AS synthesis is more strictly regulated at the posttranscriptional than the transcriptional level in vivo653046-653046
additional information?-Rattus norvegicus-involved in the recycling of L-citrulline to L-arginine653344-653344
additional information?-Homo sapiens-rate-limiting enzyme in the metabolic pathway leading from L-citrulline to L-arginine, urea cycle652976-652976
additional information?-Mus musculus, Rattus norvegicus-urea cycle, enzyme of citrulline-arginine recycling, which is important for high output production of NO in activated microglial cells, inducible enzyme653186-653186
additional information?-Mus musculus-ASS binds to and inactivates lipopolysaccharide and lipid A, overview692607--
additional information?-Homo sapiens-factors like diets, hormones and pro-inflammatory stimuli regulate ASS gene expression primarily at the transcription level690768--
additional information?-Homo sapiensP00966in mammals, the urea-cycle enzymes ASS and argininosuccinatelyase are part of an additional pathway together with the enzyme nitric oxide synthase, forming the arginine-citrulline cycle690234--
additional information?-Homo sapiens-pancreatic cancer cell lines deficient in argininosuccinate synthetase are sensitive to arginine deprivation by arginine deiminase692659--
additional information?-Homo sapiensP00966the enzyme interacts and cooperates with NADPH sensor protein, HSCARG, in downregulation of nitric oxide synthesis, the enzyme interacts with the dimerization region of HSCARG, amino acid residues 153-189, HSCARG regulation of AS activity is crucial for maintaining the intracellular balance between redox state and nitric oxide levels, overview693030--

COFACTORORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATUREIMAGE
ATPHomo sapiensP00966binding structure, conformational change upon ATP binding690234 2D-image
ATPBos taurus--690727, 690765 2D-image
ATPHomo sapiens--690768, 692659, 693030 2D-image
ATPMus musculus--692607 2D-image

METALS and IONS ORGANISM UNIPROT ACCESSION NO.COMMENTARY LITERATURE
Mg2+Thermus thermophilus-essential for catalysis652242
Mg2+Thermus thermophilusP59846required for maximal activity, in octahedral coordination with 3 oxygen atoms of the triphosphate group and 3 water molecules652404
Mg2+Mus musculus--675096, 692607
Mg2+Homo sapiens--690234, 690768, 692659, 693030
Mg2+Bos taurus--690727, 690765

INHIBITORSORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE IMAGE
1,2-CyclohexanedioneHomo sapiens-magnesiumdiphosphate and analogues protect1595 2D-image
2,3-ButanedioneHomo sapiens-magnesiumdiphosphate and analogues protect1595 2D-image
2-aminopentanoic acidRattus norvegicus-competitive to L-citrulline1580 2D-image
5,5'-dithiobis(2-nitrobenzoate)Rattus norvegicus--1576, 1582 2D-image
ADPBos taurus--1577 2D-image
alpha-methyl-D,L-aspartateRattus norvegicus--651195 2D-image
AMPBos taurus-competitive with respect to ATP1574 2D-image
AMPBos taurus--1577, 1587 2D-image
CTPRattus norvegicus-slight1576 2D-image
D-AspBos taurus--1574 2D-image
dGTPRattus norvegicus-slight1576 2D-image
diphosphateBos taurus--1574, 1587 2D-image
diphosphoryl lipid A (DPL)Mus musculus-DPL from Escherichia coli, inhibits argininosuccinate synthetase in a dose-dependent manner, concentration for half inhibition of argininosuccinate synthetase enzyme activity: 0.039 mg/ml675096-
dTTPRattus norvegicus-slight1576 2D-image
erythro-3-Hydroxy-3-methylaspartateBos taurus--1574 2D-image
GlyoxalHomo sapiens-magnesiumdiphosphate and analogues protect1595 2D-image
GTPRattus norvegicus-slight1576 2D-image
IL-1betaHomo sapiens-a long-term 24 h exposure of ASS to IL-1beta in Caco-2 cells inhibits its activity. The inhibiting effect of IL-1beta is linked to the production of nitric oxide (NO) induced by IL-1beta. The inhibiting effect is totally blocked in the presence of N-methyl-L-arginine an inhibitor of the inducible nitric oxide synthase or by culturing the cells in an arginine-deprived medium672445-
L-ArgSaccharomyces cerevisiae-non-competitive with respect to Asp and MgATP2-1579 2D-image
L-ArgRattus norvegicus-competitive to L-Asp; competitive with L-citrulline1580 2D-image
L-argininosuccinateBos taurus-competitive with respect to citrulline, Asp, and ATP1574 2D-image
L-argininosuccinateSaccharomyces cerevisiae--1579 2D-image
L-argininosuccinateRattus norvegicus-competitive to L-Asp1580 2D-image
L-argininosuccinateBos taurus--1587 2D-image
L-HisRattus norvegicus--1580 2D-image
L-IleRattus norvegicus-competitive to L-citrulline1580 2D-image
L-norvalineDaucus carota, Glomus intraradices--676636 2D-image
L-TrpRattus norvegicus--1580 2D-image
L-ValRattus norvegicus-competitive to L-citrulline1580 2D-image
MethylaspartateBos taurus--1587 2D-image
NOHomo sapiens-mediates reversible S-nitrosylation and inactivation of argininosuccinate synthetase in vitro and in lipopolysaccharide-treated cells and mice. S-nitrosylation of Cys132 is noth necessary and sufficient for the inhibition of argininosuccinate synthase by NO donors662254 2D-image
p-chloromercuribenzoatePisum sativum--1573 2D-image
PhenylglyoxalBos taurus-ATP and diphosphate protect1589 2D-image
PhenylglyoxalHomo sapiens-magnesiumdiphosphate and analogues protect1595 2D-image
phosphateSaccharomyces cerevisiae-competitive with respect to citrulline and Asp1579 2D-image
R-lipopolysaccharide (LPS)Mus musculus-rough-type LPS from Escherichia coli, inhibits argininosuccinate synthetase in a dose-dependent manner, concentration for half inhibition of argininosuccinate synthetase enzyme activity: 0.230 mg/ml675096-
recombinant mycoplasma arginine deiminaseMus musculus-36% decrease in AS activity after rADI treatment650761-
S-lipopolysaccharide (LPS)Mus musculus-smooth-type-LPS from Escherichia coli, inhibits argininosuccinate synthetase in a dose-dependent manner, concentration for half inhibition of argininosuccinate synthetase enzyme activity: 0.024 mg/ml675096-
sodium 2-deoxy-2-(3S-(9-phenyl-nonanoyl-oxy) tetradecanoyl) amino-3-O-(9-phenyl-nonanoyl)-D-glucopyranose 4-sulfateMus musculus-synthesized lipid A analog, inhibits argininosuccinate synthetase in a dose-dependent manner, concentration for half inhibition of argininosuccinate synthetase enzyme activity: 0.0065 mg/ml675096 2D-image
threo-3-Hydroxy-3-methylaspartateBos taurus--1574 2D-image
UTPRattus norvegicus-slight1576 2D-image
monophosphoryl lipid A (MPL)Mus musculus-MPL from Escherichia coli, inhibits argininosuccinate synthetase in a dose-dependent manner, concentration for half inhibition of argininosuccinate synthetase enzyme activity: 0.270 mg/ml675096-
additional informationCanis lupus familiaris-study of the effect of recombinant mycoplasma arginine deiminase on cell proliferation, correlation of the low resistance to this treatment with a low AS activity in the sensitive cell line650761-
additional informationMus musculus-study of the effect of recombinant mycoplasma arginine deiminase on cell proliferation, good correlation of the high resistance to this treatment with a high AS activity in the cell line650761-
additional informationBos taurus-1-D-ribofuranosylbenzimidazole suppresses enzyme expression690727-
additional informationHomo sapiens-HNF4 mutations influence enzyme expression activity690768-
additional informationHomo sapiens-pancreatic cancer cell lines deficient in argininosuccinate synthetase are sensitive to arginine deprivation by arginine deiminase, overview692659-

ACTIVATING COMPOUNDORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE IMAGE
IL-1betaHomo sapiens-ASS is rapidly induced by a short time exposure of IL-1beta to Caco-2 cells. By contrast, a long-term 24 h exposure to IL-1beta inhibited the ASS activity despite an increase in both specific mRNA-level and protein amount, demonstrating a post-translational effect672445-
recombinant mycoplasma arginine deiminaseHomo sapiens-350% increase in AS activity in HeLa cells after rADI treatment, no activity change in umbilical vein endothelial cells650761-
lipopolysaccharideHomo sapiens, Mus musculus-argininosuccinate synthase accumulates in circulation within 1 h after treatment with both lipopolysaccharide alone and hepatotoxic combination of lipopolysaccharide and D-Galactosamine. Enzyme physically binds to lipopolysaccharide715188 2D-image
additional informationHomo sapiens-study of the effect of recombinant mycoplasma arginine deiminase on cell proliferation, good correlation of the resistance to this treatment with the AS activity in the cell lines650761-
additional informationMus musculus-inducible enzyme, immunostimulation of AS synthesis with lipopolysaccharide or cytokines, such as TNF-alpha, interleukin 1 and interferon-gamma653046-
additional informationMus musculus-AS mRNA is 4-6fold induced by Escherichia coli lipopolysaccharide and mouse interferon-gamma, kinetics of induction653186-
additional informationRattus norvegicus-AS mRNA is induced by Escherichia coli lipopolysaccharide and mouse interferon-gamma653186-
additional informationRattus norvegicus-expression of argininosuccinate synthetase mRNA is inducible by lipopolysaccharides653344-
additional informationBos taurus-troglitazone, a PPARgamma ligand, and thiazolidinedione up-regulate vascular endothelial argininosuccinate synthase expression, quantitative real time RT-PCR, overview690727-
additional informationHomo sapiens-CRE-1 is the cis-element responsible for liver-specific cAMP induction of the human ASS gene690768-
additional informationHomo sapiens-interaction with Bj-BPP-10c, snake venom anti-hypertensive peptide of Botrops jararaca, activates the catalytic activity of argininosuccinate snythetase in a dose-dependent manner704509-
additional informationMus musculusP16460interaction with Bj-BPP-10c, snake venom anti-hypertensive peptide of Botrops jararaca, activates the catalytic activity of argininosuccinate snythetase in a dose-dependent manner704509-

KM VALUE [mM]KM VALUE [mM] MaximumSUBSTRATEORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE IMAGE
0.022-AMPRattus norvegicus--1576 2D-image
0.022-AMPRattus norvegicus-citrulline, enzyme form ASSII1582 2D-image
0.12-argininosuccinateRattus norvegicus--1576 2D-image
0.017-AspHomo sapiens--1581 2D-image
0.019-AspHomo sapiens-reaction with 5 mM citrulline1586 2D-image
0.027-AspHomo sapiens-wild-type enzyme1598 2D-image
0.078-AspHomo sapiens-MGL8B2 enzyme and MGL8D1 enzyme1583 2D-image
0.083-AspHomo sapiens-reaction with 0.5 mM citrulline1586 2D-image
1.5-AspHomo sapiens-mutant A192V1598 2D-image
4.3-AspHomo sapiens-mutant R272C1598 2D-image
0.12-aspartateMus musculusP16460in presence of 0.0005 mM Bj-BPP-10c704509 2D-image
0.17-aspartateMus musculusP16460in presence of 0.1 mM Bj-BPP-10c704509 2D-image
0.18-aspartateMus musculusP16460-704509 2D-image
0.041-ATPHomo sapiens--1581 2D-image
0.051-ATPHomo sapiens-wild-type enzyme1598 2D-image
0.077-ATPHomo sapiens-citrulline1586 2D-image
0.081-ATPHomo sapiens-mutant enzyme A192V1598 2D-image
0.11-ATPRattus norvegicus-enzyme form ASSI1582 2D-image
0.14-ATPHomo sapiens-mutant R272C1598 2D-image
0.15-ATPRattus norvegicus--1575, 1576 2D-image
0.15-ATPMus musculusP16460in presence of 0.0005 mM Bj-BPP-10c704509 2D-image
0.17-ATPRattus norvegicus-enzyme form ASSII1582 2D-image
0.18-ATPHomo sapiens-normal liver and from patients with citrullinemia1578 2D-image
0.44-ATPHomo sapiens-mutant R304W1598 2D-image
0.61-ATPMus musculusP16460in presence of 0.1 mM Bj-BPP-10c704509 2D-image
0.66-ATPMus musculusP16460-704509 2D-image
0.02-citrullineRattus norvegicus--1576 2D-image
0.02-citrullineBos taurus--1577 2D-image
0.024-citrullineHomo sapiens-reaction with 5 mM Asp1586 2D-image
0.042-citrullineHomo sapiens-reaction with 0.5 mM Asp1586 2D-image
0.044-citrullineRattus norvegicus--1575 2D-image
0.044-citrullineRattus norvegicus-Asp1576 2D-image
0.056-citrullineHomo sapiens-wild-type enzyme1598 2D-image
0.061-citrullineHomo sapiens-MGL8B2 enzyme1583 2D-image
0.089-citrullineHomo sapiens-MGL8D1 enzyme1583 2D-image
0.18-citrullineMus musculusP16460in presence of 0.0005 mM Bj-BPP-10c704509 2D-image
0.29-citrullineMus musculusP16460in presence of 0.1 mM Bj-BPP-10c704509 2D-image
0.36-citrullineMus musculusP16460-704509 2D-image
4.2-citrullineHomo sapiens-mutant R272C1598 2D-image
15-citrullineHomo sapiens-mutant A192V1598 2D-image
56-citrullineHomo sapiens-mutant R304W1598 2D-image
190-citrullineHomo sapiens-mutant R304W1598 2D-image
0.016-diphosphateRattus norvegicus--1576 2D-image
0.016-diphosphateHomo sapiens-citrulline1581 2D-image
0.025-L-AspRattus norvegicus--1575 2D-image
0.029-L-AspHomo sapiens-normal liver and from patients with citrullinemia1578 2D-image
0.035-L-AspBos taurus--1574 2D-image
0.037-L-AspRattus norvegicus-enzyme form ASSII1582 2D-image
0.038-L-AspRattus norvegicus-enzyme form ASSI1582 2D-image
0.026-L-citrullineRattus norvegicus-enzyme form ASSI1582 2D-image
4.5-threo-beta-hydroxy-L-AspBos taurus--1574 2D-image
0.88-threo-beta-methyl-L-AspBos taurus--1574 2D-image
0.03-L-citrullineHomo sapiens-normal liver1578 2D-image
additional information-additional informationHomo sapiens-ATP: biphasic double-reciprocal plot1583-
additional information-additional informationHomo sapiens--1598-

TURNOVER NUMBER [1/s] TURNOVER NUMBER MAXIMUM[1/s] SUBSTRATEORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE IMAGE
No entries in this field

kcat/KM VALUE [1/mMs-1]kcat/KM VALUE [1/mMs-1] MaximumSUBSTRATEORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE IMAGE
No entries in this field

Ki VALUE [mM]Ki VALUE [mM] MaximumINHIBITORORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE IMAGE
No entries in this field

IC50 VALUE [mM]IC50 VALUE [mM] MaximumINHIBITORORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE IMAGE
additional information-additional informationMus musculus-substrate: diphosphoryl lipid A, IC50: 0.039 mg/ml; substrate: monophosphoryl lipid A, IC50: 0.270 mg/ml; substrate: R-lipopolysaccharide, IC50: 0.230 mg/ml; substrate: S-lipopolysaccharide, IC50: 0.024 mg/ml; substrate: sodium 2-deoxy-2-(3S-(9-phenyl-nonanoyl-oxy) tetradecanoyl) amino-3-O-(p-phenyl-nonanoyl)-D-glucopyranose 4-sulfate (ONO-4007), IC50: 0.0065 mg/ml675096-

SPECIFIC ACTIVITY [µmol/min/mg] SPECIFIC ACTIVITY MAXIMUM ORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE
1e-06-Canis lupus familiaris-37°C, cell homogenate650761
6e-06-Homo sapiens-37°C, A-549 cell homogenate650761
1.3e-05-Homo sapiens-37°C, LNCaP cell homogenate650761
1.9e-05-Homo sapiens-37°C, MCF-7 cell homogenate650761
4.8e-05-Homo sapiens-37°C, HeLa cell homogenate650761
0.000174-Mus musculus-37°C, cell homogenate650761
0.002-Daucus carota-in the presence of 5 mM (NH4)2SO4 and after addition of 20 mM L-norvaline in root-free compartments676636
0.0028-Glomus intraradices-in the presence of 5 mM (NH4)2SO4 and after addition of 20 mM L-norvaline in the root-free compartments676636
0.0071-Daucus carota-in the presence of 5 mM (NH4)2SO4 and after addition of 0.5 mM phenyl phosphorodiamidate in root-free compartments676636
0.0085-Daucus carota-in the presence of 5 mM (NH4)2SO4 in root-free compartments676636
0.0166-Daucus carota-in the absence of (NH4)2SO4 in root-free compartments676636
0.0166-Glomus intraradices-in the absence of (NH4)2SO4 in the root-free compartments676636
0.049-Glomus intraradices-in the presence of 5 mM (NH4)2SO4 and after addition of 0.5 mM phenyl phosphorodiamidate in the root-free compartments; in the presence of 5 mM (NH4)2SO4 in the root-free compartments676636
0.06-Thermus thermophilusP5984620°C652404
0.14-Mus musculus-purification step: liver homogenate supernatant675096
0.27-Mus musculus-purification step: 45-55% saturated ammonium sulfate precipitate675096
0.92-Thermus thermophilusP5984670°C652404
0.95-Homo sapiens--1583
0.99-Mus musculus-purification step: DE-52 fraction675096
1-Homo sapiens-specific activity of ASS in Caco-2 cells after long time exposure (24h) to 1 ng/ml IL-1beta672445
1.27-Homo sapiens-specific activity of ASS in Caco-2 cells after the addition of 0.5 mM S-nitroso-acetylpenicillamine for 24h, an NO donor. This clearly demonstrates that NO induces an inhibition of the ASS activity in Caco-2 cells.672445
1.3-Homo sapiens--1586
1.39-Mus musculus-purification step: S-300 fraction675096
2.1-Rattus norvegicus--1575
2.5-Homo sapiens-specific activity of ASS in Caco-2 cells after short time exposure (4h) to 1 ng/ml IL-1beta672445
3.8-Bos taurus--1585
3.83-Bos taurus--1574, 1577
4.2-Homo sapiens--1581
4.54-Saccharomyces cerevisiae--1579
5.16-Mus musculus-purification step: UNO S-1 fraction675096
12.8-Pisum sativum--1573

pH OPTIMUMpH MAXIMUMORGANISM UNIPROT ACCESSION NO. COMMENTARYLITERATURE
66.5Saccharomyces cerevisiae-reaction of AMP + diphosphate + L-argininosuccinate1579
6-Bos taurus-reaction of AMP + diphosphate + L-argininosuccinate1574
6.26.3Rattus norvegicus-reaction of AMP + diphosphate + L-argininosuccinate1576
7.57.8Saccharomyces cerevisiae-reaction of ATP + citrulline + L-argininosuccinate1579
7.5-Mus musculus-assay at675096
7.5-Daucus carota, Glomus intraradices-assay at676636
7.8-Homo sapiens-assay at693030
7.8-Mus musculusP16460assay at704509
7.9-Pisum sativum--1573
8.48.8Rattus norvegicus--1575
8.4-Rattus norvegicus-reaction of ATP + citrulline + L-Asp1576
8.5-Thermus thermophilusP59846assay at652404
8.6-Rattus norvegicus-reaction of ATP + citrulline + L-Asp, enzyme forms ASSI and ASSII1582
8.7-Bos taurus-reaction of ATP + L-citulline + L-Asp1574

pH RANGEpH RANGE MAXIMUMORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
5.57Saccharomyces cerevisiae-about 60% of maximal activity at pH 5.5 and 71579
6.58.5Saccharomyces cerevisiae-6.5: about 55% of maximal activity, 8.5: about 70% of maximal activity, reaction of ATP + citrulline + L-argininosuccinate1579

TEMPERATURE OPTIMUMTEMPERATURE OPTIMUM MAXIMUMORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
26-Daucus carota, Glomus intraradices-assay at676636
37-Canis lupus familiaris, Homo sapiens, Mus musculus-assay at650761
37-Homo sapiens-assay at672445
37-Mus musculus-assay at675096
37-Homo sapiens-assay at693030
37-Mus musculusP16460assay at704509

TEMPERATURE RANGE TEMPERATURE MAXIMUM ORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE
No entries in this field

pI VALUEpI VALUE MAXIMUMORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
No entries in this field

SOURCE TISSUE ORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE SOURCE
adenocarcinoma cell lineHomo sapiens-human adenocarcinoma cell line Caco-2/TC7672445Manually annotated by BRENDA team
aortaBos taurus-endothelial cell662377Manually annotated by BRENDA team
aortaBos taurus--690727, 690765Manually annotated by BRENDA team
bloodHomo sapiens--703248Manually annotated by BRENDA team
bloodMus musculus--715188Manually annotated by BRENDA team
brainRattus norvegicus--1594Manually annotated by BRENDA team
BxPC-3 cellHomo sapiens-pancreatic cancer cell line, low ASS expression level692659Manually annotated by BRENDA team
Caco-2/TC7 cellHomo sapiens--661285Manually annotated by BRENDA team
capAgaricus bisporusQ2Z0F6Northern analyses of ass during fruiting body formation and post-harvest development reveals that expression is significantly up-regulated from developmental stage 3 on for all the tissues studied (gills, stip and cap). The expression reaches a maximum at the later stages of fruiting body growth, stages 6 and 7676120Manually annotated by BRENDA team
Capan-1 cellHomo sapiens-pancreatic cancer cell line, low ASS expression level692659Manually annotated by BRENDA team
carcinoma cell lineHomo sapiens--704078Manually annotated by BRENDA team
corpus striatumRattus norvegicus-in a rat ischemia model the striatum is examined at 24, 72 and 144 h after reperfusion and compared with rats in normal conditions. At 24 h after reperfusion, the number of the nitric oxide synthase-positive neurons and the percentage of those co-expressing argininosuccinate synthetase and argininsuccinate lyase and nitric oxide synthase are significantly increased in the animals with a longer survival673486Manually annotated by BRENDA team
cotyledonPisum sativum--1573Manually annotated by BRENDA team
endothelial cellBos taurus--662205Manually annotated by BRENDA team
endothelial cellBos taurus-aorta662377Manually annotated by BRENDA team
endothelial cellBos taurus-aortic690727, 690765Manually annotated by BRENDA team
endothelial cellHomo sapiens--704078Manually annotated by BRENDA team
endothelial cell lineBos taurus-expression analysis is performed in bovine aortic endothelial cells (BAEC)671250Manually annotated by BRENDA team
epithelial cellHomo sapiens--693030Manually annotated by BRENDA team
gillAgaricus bisporusQ2Z0F6Northern analyses of ass during fruiting body formation and post-harvest development reveals that expression is significantly up-regulated from developmental stage 3 on for all the tissues studied (gills, stip and cap). The expression reaches a maximum at the later stages of fruiting body growth, stages 6 and 7676120Manually annotated by BRENDA team
HEK-293 cellHomo sapiens--704509Manually annotated by BRENDA team
hepatocyteMus musculus--692607Manually annotated by BRENDA team
HPAC cellHomo sapiens-pancreatic cancer cell line, low ASS expression level692659Manually annotated by BRENDA team
HPAF-2 cellHomo sapiens-pancreatic cancer cell line, low ASS expression level692659Manually annotated by BRENDA team
Huh-7 cellHomo sapiens-a human hepatoma cell line690768Manually annotated by BRENDA team
HUVEC cellHomo sapiens--704509, 715624Manually annotated by BRENDA team
hypothalamusFelis catus--1590Manually annotated by BRENDA team
inflammatory cellHomo sapiens--704078Manually annotated by BRENDA team
kidneyBos taurus--1574Manually annotated by BRENDA team
kidneySus scrofa-proximal tubules1596Manually annotated by BRENDA team
kidneyHomo sapiens--704078Manually annotated by BRENDA team
kidneyMus musculusP16460-704509Manually annotated by BRENDA team
liverBos taurus--1574, 1577, 1585, 1587, 1589Manually annotated by BRENDA team
liverRattus norvegicus--1575, 1576, 1580, 1582, 1592, 1597Manually annotated by BRENDA team
liverHomo sapiens-normal and from patients with citrullinemia1578Manually annotated by BRENDA team
liverHomo sapiens--1581, 690768, 704078, 705506Manually annotated by BRENDA team
liverMus musculus--675096, 692607, 715188Manually annotated by BRENDA team
lymphoblastHomo sapiens-parental cell line MGL8B2 and canavanine-resistant cell line MGL8D11583Manually annotated by BRENDA team
myceliumGlomus intraradices-extraradical myecelium676636Manually annotated by BRENDA team
non-small cell lung cancer cellHomo sapiens-increased co-expression of AS and TNF-a mRNA is observed in non-small cell lung and stomach cancer, compared with normal corresponding tissues674048Manually annotated by BRENDA team
ovary cancer cellHomo sapiens-AS mRNA increases 2- to 3fold by 24 h in both the IGROV-1 cell line, and a second ovarian cancer cell line OVCAR-3, following treatment with TNF-alpha. In contrast, although the ovarian cancer cell lines, PEO1 and SKOV-3, express AS in abundance, this is not TNF-a-inducible; TNF-alpha induces prolonged expression of AS mRNA and protein in ovarian tumour cells, contrasting with short-lived expression of AS mRNA in the normal ovarian epithelial cells. AS mRNA is higher expressed in ovarian tumor tissue compared to normal ovarian tissue. Moreover, AS protein colocalises with TNF-alpha in ovarian cancer cells, with significantly higher levels of AS in malignant compared with normal ovarian tissue.674048Manually annotated by BRENDA team
ovary cancer cell lineHomo sapiens--704070Manually annotated by BRENDA team
OVCA-433 cellHomo sapiens--704070Manually annotated by BRENDA team
PANC-1 cellHomo sapiens-pancreatic cancer cell line, very low ASS expression level692659Manually annotated by BRENDA team
pancreatic cancer cell lineHomo sapiens-pancreatic cancer cell line, low ASS expression level692659Manually annotated by BRENDA team
plasmaHomo sapiens--703251Manually annotated by BRENDA team
rootDaucus carota-AM roots676636Manually annotated by BRENDA team
RPMI-2650 cellHomo sapiens-a human epithelium cell line690768Manually annotated by BRENDA team
serumHomo sapiens-argininosuccinate synthase accumulates in circulation within 1 h after treatment with both lipopolysaccharide alone and hepatotoxic combination of lipopolysaccharide and D-Galactosamine715188Manually annotated by BRENDA team
SKOV-3 cellHomo sapiens--704070Manually annotated by BRENDA team
stipeAgaricus bisporusQ2Z0F6Northern analyses of ass during fruiting body formation and post-harvest development reveals that expression is significantly up-regulated from developmental stage 3 on for all the tissues studied (gills, stip and cap). The expression reaches a maximum at the later stages of fruiting body growth, stages 6 and 7676120Manually annotated by BRENDA team
stomachHomo sapiens-increased co-expression of AS and TNF-a mRNA is observed in non-small cell lung and stomach cancer, compared with normal corresponding tissues674048Manually annotated by BRENDA team
testisRattus norvegicus--1597Manually annotated by BRENDA team
urineHomo sapiens--703251Manually annotated by BRENDA team
MiaPaCa-2 cellHomo sapiens-pancreatic cancer cell line, very low ASS expression level692659Manually annotated by BRENDA team
additional informationBos taurus-treatment of BAEC cells with tumor necrosis factor-alpha supresses argininosuccinate synthase expression. reporter gene analysis demonstrates that TNF-alpha suppresses the AS proximal promoter and electrophoretic mobility shift assay shows reduced binding to three essential Sp1 elements671250Manually annotated by BRENDA team
additional informationAgaricus bisporusQ2Z0F6ASS is up-regulated within 3 h after harvest showing that the induction mechanism is very sensitive to the harvest event and emphasizes the importance of the arginine biosynthetic pathway/ornithine cycle in post-harvest physiology676120Manually annotated by BRENDA team
additional informationHomo sapiens-87% of human tumors and 5 of 7 cancer cell lines lack ASS expression, overview692659Manually annotated by BRENDA team

LOCALIZATION ORGANISM UNIPROT ACCESSION NO. COMMENTARY GeneOntology No. LITERATURE SOURCE
caveolaHomo sapiens--5901704078Manually annotated by BRENDA team
cytoplasmBos taurus--57371585Manually annotated by BRENDA team
cytoplasmRattus norvegicus--57371591Manually annotated by BRENDA team
cytoplasmRattus norvegicus-the enzyme is mainly mitochondrial in fetal and new-born liver, and cytoplasmic in adult liver57371592Manually annotated by BRENDA team
cytoplasmSus scrofa--57371596Manually annotated by BRENDA team
cytosolRattus norvegicus--58291591Manually annotated by BRENDA team
cytosolMus musculus--5829692607, 704509Manually annotated by BRENDA team
cytosolHomo sapiens--5829704078Manually annotated by BRENDA team
mitochondrial outer membraneRattus norvegicus-outer membrane57411591Manually annotated by BRENDA team
mitochondrial outer membraneRattus norvegicus-outer membrane; the enzyme is mainly mitochondrial in fetal and new-born liver, and cytoplasmic in adult liver57411592Manually annotated by BRENDA team
additional informationRattus norvegicus-at the activity level, the colocation of argininosuccinate synthetase is changing during fetal and neonatal developement and is under the control of corticosteroid and pancreatic hormones-1591Manually annotated by BRENDA team

PDBSCOPCATHORGANISM
1k92, downloadSCOP (1k92)CATH (1k92)Escherichia coli (strain K12)
1k97, downloadSCOP (1k97)CATH (1k97)Escherichia coli (strain K12)
1kp2, downloadSCOP (1kp2)CATH (1kp2)Escherichia coli (strain K12)
1kp3, downloadSCOP (1kp3)CATH (1kp3)Escherichia coli (strain K12)
2nz2, downloadSCOP (2nz2)CATH (2nz2)Homo sapiens
1vl2, downloadSCOP (1vl2)CATH (1vl2)Thermotoga maritima (strain ATCC 43589 / MSB8 / DSM 3109 / JCM 10099)
1j1z, downloadSCOP (1j1z)CATH (1j1z)Thermus thermophilus (strain HB8 / ATCC 27634 / DSM 579)
1j20, downloadSCOP (1j20)CATH (1j20)Thermus thermophilus (strain HB8 / ATCC 27634 / DSM 579)
1j21, downloadSCOP (1j21)CATH (1j21)Thermus thermophilus (strain HB8 / ATCC 27634 / DSM 579)
1kh1, downloadSCOP (1kh1)CATH (1kh1)Thermus thermophilus (strain HB8 / ATCC 27634 / DSM 579)
1kh2, downloadSCOP (1kh2)CATH (1kh2)Thermus thermophilus (strain HB8 / ATCC 27634 / DSM 579)
1kh3, downloadSCOP (1kh3)CATH (1kh3)Thermus thermophilus (strain HB8 / ATCC 27634 / DSM 579)
1kor, downloadSCOP (1kor)CATH (1kor)Thermus thermophilus (strain HB8 / ATCC 27634 / DSM 579)

MOLECULAR WEIGHT MOLECULAR WEIGHT MAXIMUM ORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE
45000-Mus musculus-SDS-PAGE675096
46000-Mus musculusP16460SDS-PAGE704509
47270-Agaricus bisporusQ2Z0F6deduced from cDNA676120
48500-Porphyra yezoensisQ0KKQ0deduced from cDNA675606
80000-Sus scrofa-gel filtration1596
158000-Rattus norvegicus-gel filtration1576
175000-Bos taurus-gel filtration1574
180000-Homo sapiens-gel filtration1583, 1598
183000-Homo sapiens-gel filtration1581
185000-Bos taurus-gel filtration1577
185000-Homo sapiens-sucrose density gradient centrifugation1578
185000-Bos taurus-gel filtration1585
185000-Homo sapiens-gel filtration1586
207000-Rattus norvegicus-sedimentation equilibrium centrifugation1576
228000-Saccharomyces cerevisiae-gel filtration1579

SUBUNITS ORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE
?Rattus norvegicus-x * 48000, SDS-PAGE1575, 1582
?Mus musculus-x * 46000, immunoblot analysis653186
?Homo sapiens, Mus musculus-x * 47000, calculated for His-tagged recombinant protein715188
dimerSus scrofa-2 * 45000, SDS-PAGE under reducing and nonreducing conditions1596
homotetramerEscherichia coli-4 * 50000, SDS-PAGE649104
homotetramerEscherichia coli-each monomer of tetrameric EAS consits of a nucleotide binding domain and a novel catalytic/multimerization domain, subunit/domain structure, subunit interactions652231
homotetramerRattus norvegicus-4 * 45400, Western blot analysis, calculation from the amino acid sequence653344
tetramerBos taurus-2 * 90000, SDS-PAGE in absence of reducing agent; 4 * 45000, two pairs of monomers crosslinked by disulfide bonds, SDS-PAGE in presence of reducing agent1574
tetramerRattus norvegicus-4 * 48000, SDS-PAGE, PAGE with 6 M urea1576
tetramerBos taurus-4 * 46500, SDS-PAGE in presence of 2-mercaptoethanol1577, 1585
tetramerSaccharomyces cerevisiae-, 4 * 49000, SDS-PAGE in presence of 2-mercaptoethanol1579
tetramerHomo sapiens-4 * 43000, SDS-PAGE1581
tetramerHomo sapiens-4 * 45000, SDS-PAGE1583, 1586
tetramerRattus norvegicus--1586
tetramerHomo sapiens-4 * 46000, SDS-PAGE1598
tetramerThermus thermophilus-4 * 44815, recombinant tAsS, subunit/domain structure, amino acid sequence652242, 652404
tetramerHomo sapiensP00966two dimers, dimeric and tetrameric interface structures, overview690234

POSTTRANSLATIONAL MODIFICATION ORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE
nitrosylationHomo sapiens-exposure of ASS to IL-1beta in Caco-2 cells induces the nitrosylation of the ASS protein672445
phosphoproteinBos taurus-regulation of argininosuccinate synthase by dynamic phosphorylation through protein kinase A, the enzyme lacks a consensus Akt phosphorylation motif R-X-R-X-X-S/T690765
additional informationHomo sapiens-it is shown that IL-1beta inhibits the ASS activity through a post-translational mechanism since increase in both specific mRNA-level and protein amount can be detected after long-time exposure of Caco-2 cells to IL-1beta. The inhibiting effect of IL-1beta is linked to the production of nitric oxide (NO) induced by IL-1beta672445

Crystallization/COMMENTARY ORGANISM UNIPROT ACCESSION NO. LITERATURE
-Bos taurus-1574, 1577, 1585
crystal strucures in complex with intact ATP and with ATP and citrulline, hanging drop vapour-diffusion technique, X-ray analysisEscherichia coli-652231
recombinant EAS, hanging drop vapour-diffusion technique, X-ray analysisEscherichia coli-649104
purified recombinant ASS1 by hanging-drop method, 0.001 ml of protein solution containing 17 mg/ml protein in 500 mM NaCl, 2 mM TCEP, 30 mM HEPES, pH 7.5, 10 mM aspartate, and 10 mM citrulline, is mixed with 0.001 ml of reservoir solution containing 16% w/v PEG 3350, 0.15 M dl-malic acid pH 7.0, X-ray diffraction structure determination and analysis at 2.4-2.5 A resolutionHomo sapiensP00966690234
-Rattus norvegicus-1575, 1576, 1580
3-dimensional structure of free enzyme and of complexes with intact ATP, adenylyl imidodiphosphate, arginine or succinate, hanging drop methodThermus thermophilus-652242
structures of enzyme complexed with intact ATP and substrates citrulline and aspartate, complexed with AMP and argininosuccinate, and complexed with AMP-PNP, arginine, aspartate and Mg2+, vapor diffusion method, X-ray analysisThermus thermophilusP59846652404

pH STABILITYpH STABILITY MAXIMUM ORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE
No entries in this field

TEMPERATURE STABILITYTEMPERATURE STABILITY MAXIMUM ORGANISM UNIPROT ACCESSION NO. COMMENTARYLITERATURE
45-Rattus norvegicus-enzyme forms ASSI and II are stable, enzyme form ASSIII is very unstable1582
50-Homo sapiens-7 min, enzyme from normal liver and from patients with citrullinemia, in presence of 10 mM argininosuccinate, about 10% loss of activity; 7 min, enzyme from normal liver, in absence of argininosuccinate, about 65% loss of activity; 7 min, enzyme from patients with citrullinemia, in absence of argininosuccinate, 95% loss of activity1578
additional information-Homo sapiens-argininosuccinate stabilizes against heat treatment1578

GENERAL STABILITYORGANISM UNIPROT ACCESSION NO.LITERATURE
unstable during purificationBos taurus-1585
argininosuccinate stabilizes against heat treatmentHomo sapiens-1578
in presence of 5 mM argininosuccinate, the activities of the three forms of the enzyme are resistant to chymotrypsinRattus norvegicus-1582

ORGANIC SOLVENT ORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE
No entries in this field

OXIDATION STABILITY ORGANISM UNIPROT ACCESSION NO. LITERATURE
No entries in this field

STORAGE STABILITY ORGANISM UNIPROT ACCESSION NO. LITERATURE
-70°C, stable for 3 monthsBos taurus-1577
quite stable frozen or as ammonium sulfate precipitateHomo sapiens-1581
-20°C, 0.05 M citrulline, 0.05 M Asp, or 0.01 M argininosuccinate, stable for more than 1 yearRattus norvegicus-1576
-20°C, stable for monthsSaccharomyces cerevisiae-1579
0-4°C, without (NH4)2SO4, stable for about 2 weeksSaccharomyces cerevisiae-1579

Purification/COMMENTARY ORGANISM UNIPROT ACCESSION NO. LITERATURE
-Bos taurus-1577
recombinant His-tagged enzyme from Escherichia coli by nickel affinty chromatographyBos taurus-690765
recombinant EASEscherichia coli-649104, 652231
-Homo sapiens-1581, 1583, 1586, 1598
affinity chromatographyHomo sapiens-704509
recombinantHomo sapiens-662254
recombinant ASS1 by His affinity chromatography and gel filtrationHomo sapiensP00966690234
affinity chromatographyMus musculusP16460704509
analysis by native gel electrophoresis shows that argininosuccinate synthetase can bind bacterial lipopolysaccharide and lipid A; argininosuccinate synthetase is purified from mouse liver. Livers are excised from 20 male mice (C3H/He strain, age 7-8 weeks) and homogenized in 100 mM Tris-HCl (pH 7.5) containing 1 mM citrulline and 1 mM aspartic acid. Homogenate is centrifuged. After salting-out with (NH4)2SO4. The sample is subjected to anion-exchange chromatography on DE-52. Concentrated samples are loaded on a column of Sephacryl S-300 HR. The collected first peak fractions are dialyzed and subjected to anion-exchange chromatography on UNO S-1Mus musculus-675096
-Pisum sativum-1573
-Rattus norvegicus-1575, 1597
3 enzyme forms ASSI, ASSII, and ASSIIIRattus norvegicus-1582
-Saccharomyces cerevisiae-1579
-Sus scrofa-1596
-Thermus thermophilus-652242, 652404

Cloned/COMMENTARY ORGANISM UNIPROT ACCESSION NO. LITERATURE
cloned in Escherichia coliAgaricus bisporusQ2Z0F6676120
expression analysis, the AS gene promoter contains a a near consensus PPARgamma response element, PPRE, overviewBos taurus-690727
expression of the His-tagged enzyme in Escherichia coliBos taurus-690765
expression in Escherichia coli BB101Escherichia coli-649104, 652231
expressed as a C-terminal fusion protein with maltose-binding protein in Escherichia coliHomo sapiens-662254
expression alone or together with FLAG-tagged HSCARG or with HSCARG siRNA in HeLa and HEK-293 cells, expression analysis, overviewHomo sapiens-693030
expression in Escherichia coliHomo sapiens-715188
expression of ASS1Homo sapiensP00966690234
-Mus musculus-653046
expression in Escherichia coliMus musculus-715188
the gene encoding argininosuccinate synthetase of Porphyra yezoensis is obtained by PCR using an expressed sequence tag clone as a template, and subcloned into the yeast expression vector pYES2. The gene is expressed when the vector harboring PyARG1 is introduced into an ARG1-deficient strain of Saccharomyces cerevisiae, which results in complementation of the mutant phenotype. The transformed cells survive on a selective medium lacking arginine, and transcripts of PyARG1 are detected by RT-PCR. A quantitative comparison shows that the rescued mutant cells grow in the selective liquid medium with a minor reduction in growth rate relative to wild-type cellsPorphyra yezoensisQ0KKQ0675606
expression in Escherichia coli as fusion protein with maltose binding proteinRattus norvegicus-1597
expression in Escherichia coli BL21(DE3)pLysEThermus thermophilusP59846652404
overexpression in Escherichia coliThermus thermophilus-652242

EXPRESSION ORGANISM UNIPROT ACCESSION NO. LITERATURE
tumoural enzyme downregulation by hepatocellular carcinoma, malignant melanoma, malignant pleural mesothelioma, prostate and renal cancerHomo sapiens-704078
downregulation of enzyme expression is associated with the development of platinum resistance to platinum-based drugsHomo sapiens-704070
hepatic argininosuccinate synthase expression is hormonally regulated, expression of endothelial and inflammatory cell argininosuccinate synthase is under control of cytokinesHomo sapiens-704078
overexpression of argininosuccinate synthase in primary ovarian, stomach and colorectal cancer cells results in platinum sensitive tumoursHomo sapiens-704078
increased expression in drug resistant mutantLeishmania infantumA4I066703594

ENGINEERINGORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
A192VHomo sapiens-mutant G280R has no ASS activity, mutants A192V, R272C, and R304W have low ASS activity and abnormal kinetics. A higher Km for citrulline than in wild-type enzyme is obtained with mutants A192V, R272C, and R304W1598
D296GHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
E191QHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
G1168AHomo sapiens-mutation results in citrullinemia type I703248
G156delHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
G280RHomo sapiens-mutant G280R has no ASS activity, mutants A192V, R272C, and R304W have low ASS activity and abnormal kinetics. A higher Km for citrulline than in wild-type enzyme is obtained with mutants A192V, R272C, and R304W1598
G324VHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
G347RHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
K277THomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
L160PHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
N158delHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
P96HHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
Q311delHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
Q401delHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
R127QHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
R127WHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
R265CHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
R272CHomo sapiens-mutant G280R has no ASS activity, mutants A192V, R272C, and R304W have low ASS activity and abnormal kinetics. A higher Km for citrulline than in wild-type enzyme is obtained with mutants A192V, R272C, and R304W1598
R304WHomo sapiens-mutant G280R has no ASS activity, mutants A192V, R272C, and R304W have low ASS activity and abnormal kinetics. A higher Km for citrulline than in wild-type enzyme is obtained with mutants A192V, R272C, and R304W1598
R344delHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
S341FHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
S79PHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
T284IHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
Y291SHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
Y359DHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
additional informationAgaricus bisporusQ2Z0F6an alignment with ASS proteins from other organisms reveals high similarity with fungal (61–63%) and mammalian (51–55%) ASS proteins676120
L206PHomo sapiens-mutation of the argininosuccinate synthetase gene, found in patients703969
additional informationHomo sapiensP00966clinical mutations, phenotypes, overview690234
additional informationHomo sapiens-enzyme overexpression induces a relocalization of HSCARG693030

Renatured/COMMENTARYORGANISM UNIPROT ACCESSION NO.LITERATURE
No entries in this field

APPLICATIONORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
medicineBos taurus-the downregulation of tumor necrosis factor-alpha is an added insult to endothelial function because of its important role in NO production and in endothelial viability671250
drug developmentHomo sapiens-high levels of AS expression, which may be required for several arginine-dependent processes in cancer, including the production of nitric oxide, proline, pyrimidines and polyamines, is regulated by TNF-alpha and may provide an important molecular pathway linking inflammation and metabolism to ovarian tumorigenesis674048
medicineHomo sapiens-neuronal and glial ASS expression is increased in brains of patients with Alzheimer disease652976
medicineHomo sapiens-a case of late-onset CTLN1 (citrullinemia) in a patient is described by biochemical analyses and ASS gene mutation confirmation. This is the first report of a Korean patient with late-onset CTLN1 confirmed by ASS gene mutation identification674383
medicineHomo sapiens-potential novel therapeutic strategies for ovarian cancer704070
medicineHomo sapiens-potential target in platinum sensitive tumours704078
medicineHomo sapiens-novel target for the therapy of hypertension-related disorders704509
medicineMus musculus-AS and GTP cyclohydrolase I are intimately associated with inflammatory arthritis, they may be important modulators of arthritis and may represent novel targets for modulation of disease activity653046
medicineMus musculus-citrulline-arginine recycling enzyme, intervention in the supply of arginine may represent a new therapeutic approach to the treatment of NO-mediated neurodegenerative damage in the brain653186
medicineMus musculus-argininosuccinate synthetase is inhibited by endotoxin, implying that bacterial products might potentially regulate the arginine metabolism in the host and perturb NO synthesis675096
medicineMus musculusP16460novel target for the therapy of hypertension-related disorders704509
molecular biologyPorphyra yezoensisQ0KKQ0report in which the function of a Porphyra yezoensis gene has been directly demonstrated by the rescue of a Saccharomyces cerevisiae mutant. This technique may provide new opportunities for further investigations into the functions of various genes in Porphyra yezoensis and other macroalgal species675606
medicineRattus norvegicus-transient ischemia induces changes in nitric oxide synthase, argininosuccinate synthetase and argininsuccinate lyase co-expression in the rat striatal neurons. At 24 h after reperfusion in the animals with a longer survival and better motor-sensory performances there is an increase in these neuronal populations. It is hypothesized that the elevated expression of argininosuccinate synthetase and argininsuccinate lyase protects ischemic striatal neurons from tissue damages by maintaining a correct NO production673486

DISEASETITLE OF PUBLICATIONLINK TO PUBMED
Alzheimer DiseaseNeuronal and glial coexpression of argininosuccinate synthetase and inducible nitric oxide synthase in Alzheimer disease. PubMed
amino-acid n-acetyltransferase deficiencyGenetic approach to prenatal diagnosis in urea cycle defects. PubMed
Amyloid Neuropathies, Familial[Recent advances of the treatment in metabolic disorders] PubMed
arginase deficiencyGenetic approach to prenatal diagnosis in urea cycle defects. PubMed
argininosuccinate lyase deficiencyComplementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts. PubMed
argininosuccinate lyase deficiencyGenetic approach to prenatal diagnosis in urea cycle defects. PubMed
argininosuccinate lyase deficiencyProspective treatment of urea cycle disorders. PubMed
argininosuccinate lyase deficiencyStudies on complementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts. PubMed
argininosuccinate synthase deficiencyA GC/MS-based metabolomic approach for diagnosing citrin deficiency. PubMed
argininosuccinate synthase deficiencyA novel inborn error of metabolism detected by elevated methionine and/or galactose in newborn screening: neonatal intrahepatic cholestasis caused by citrin deficiency. PubMed
argininosuccinate synthase deficiencyA patient with type II citrullinemia who developed refractory complex seizure. PubMed
argininosuccinate synthase deficiencyAn adult-onset case of argininosuccinate synthetase deficiency presenting with atypical citrullinemia. PubMed
argininosuccinate synthase deficiencyArgininosuccinate synthetase deficiency and reye syndrome-like presentation. PubMed
argininosuccinate synthase deficiencyArgininosuccinate synthetase deficiency: mutation analysis in 3 Thai patients. PubMed
argininosuccinate synthase deficiencyArgininosuccinic acid synthetase deficiency in a hamster cell line and its complementation of argininosuccinic aciduria human fibroblasts. PubMed
argininosuccinate synthase deficiencyCitrullinemia, argininosuccinate synthetase deficiency. Repository identification No. GM-1044. PubMed
argininosuccinate synthase deficiencyClearance of amino acids by hemodialysis in argininosuccinate synthetase deficiency. PubMed
argininosuccinate synthase deficiencyClinical and biochemical characteristics of patients with urea cycle disorders in a developing country. PubMed
argininosuccinate synthase deficiencyEffects of glucagon on urinary excretion of urea and on plasma ammonia level in argininosuccinate synthetase deficiency. PubMed
argininosuccinate synthase deficiencyGenetic approach to prenatal diagnosis in urea cycle defects. PubMed
argininosuccinate synthase deficiencyIn vivo urea cycle flux distinguishes and correlates with phenotypic severity in disorders of the urea cycle. PubMed
argininosuccinate synthase deficiencyIncidence and distribution of argininosuccinate synthetase deficiency in human cancers: a method for identifying cancers sensitive to arginine deprivation. PubMed
argininosuccinate synthase deficiencyInfantile cholestatic jaundice associated with adult-onset type II citrullinemia. PubMed
argininosuccinate synthase deficiencyLetter: The high levels of lysine, homocitrulline, and homoarginine found in argininosuccinate synthetase deficiency. PubMed
argininosuccinate synthase deficiencyLetter: The presence of the homoanalogues of substrates of the urea cycle in the presence of argininosuccinate synthetase deficiency. PubMed
argininosuccinate synthase deficiencyLiving-related liver transplantation for type II citrullinemia using a graft from heterozygote donor. PubMed
argininosuccinate synthase deficiencyLong-term survival of patients with argininosuccinate synthetase deficiency. PubMed
argininosuccinate synthase deficiencyMild citrullinemia in Caucasians is an allelic variant of argininosuccinate synthetase deficiency (citrullinemia type 1). PubMed
argininosuccinate synthase deficiencyMolecular analysis of argininosuccinate synthetase deficiency in human fibroblasts. PubMed
argininosuccinate synthase deficiencyMolecular definition of bovine argininosuccinate synthetase deficiency. PubMed
argininosuccinate synthase deficiencyNeonatal presentation of adult-onset type II citrullinemia. PubMed
argininosuccinate synthase deficiencyNeonatal type of argininosuccinate synthetase deficiency. Report of two cases with autopsy findings. PubMed
argininosuccinate synthase deficiencyOrthotopic liver transplantation for urea cycle enzyme deficiency. PubMed
argininosuccinate synthase deficiencyPlasma concentrations and renal clearance of orotic acid in argininosuccinic acid synthetase deficiency. PubMed
argininosuccinate synthase deficiencyPostpartum 'psychosis' in mild argininosuccinate synthetase deficiency. PubMed
argininosuccinate synthase deficiencyPregnancy in a healthy woman with untreated citrullinemia. PubMed
argininosuccinate synthase deficiencyPrenatal diagnosis of citrullinemia and argininosuccinic aciduria: evidence for a transmission ratio distortion in citrullinemia. PubMed
argininosuccinate synthase deficiencyProspective treatment of urea cycle disorders. PubMed
argininosuccinate synthase deficiencyStudies on complementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts. PubMed
argininosuccinate synthase deficiencyTransiently reduced activity of carbamyl phosphate synthetase and ornithine transcarbamylase in liver of children with Reye's syndrome. PubMed
argininosuccinate synthase deficiencyUrea cycle disorders in Thai infants: a report of 5 cases. PubMed
argininosuccinate synthase deficiency[Argininosuccinate synthetase deficiency] PubMed
Argininosuccinic AciduriaArgininosuccinic acid synthetase deficiency in a hamster cell line and its complementation of argininosuccinic aciduria human fibroblasts. PubMed
Argininosuccinic AciduriaComplementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts. PubMed
Argininosuccinic AciduriaGenetic approach to prenatal diagnosis in urea cycle defects. PubMed
Argininosuccinic AciduriaProspective treatment of urea cycle disorders. PubMed
Argininosuccinic AciduriaStudies on complementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts. PubMed
ArthritisInduction of inducible nitric oxide synthase, argininosuccinate synthase, and GTP cyclohydrolase I in arthritic joints of human tumor necrosis factor-alpha transgenic mice. PubMed
Bacterial InfectionsInhibition of LPS toxicity by hepatic argininosuccinate synthase (ASS): Novel roles for ASS in innate immune responses to bacterial infection. PubMed
carbamoyl-phosphate synthase (ammonia) deficiencyIncreased excretion of N-carbamoyl compounds in patients with urea cycle defects. PubMed
carbamoyl-phosphate synthase (ammonia) deficiencyProspective treatment of urea cycle disorders. PubMed
CarcinomaRenal cell carcinoma does not express argininosuccinate synthetase and is highly sensitive to arginine deprivation via arginine deiminase. PubMed
Carcinoma, HepatocellularA randomised phase II study of pegylated arginine deiminase (ADI-PEG 20) in Asian advanced hepatocellular carcinoma patients. PubMed
Carcinoma, HepatocellularArginine deprivation, growth inhibition and tumour cell death: 3. Deficient utilisation of citrulline by malignant cells. PubMed
Carcinoma, HepatocellularCombined lysosomal protein transmembrane 4 beta-35 and argininosuccinate synthetase expression predicts clinical outcome in hepatocellular carcinoma patients. PubMed
Carcinoma, HepatocellularPegylated recombinant human arginase (rhArg-peg5,000mw) inhibits the in vitro and in vivo proliferation of human hepatocellular carcinoma through arginine depletion. PubMed
Carcinoma, HepatocellularPhase II study of pegylated arginine deiminase for nonresectable and metastatic hepatocellular carcinoma. PubMed
Carcinoma, HepatocellularReduced expression of ASS is closely related to clinicopathological features and post-resectional survival of hepatocellular carcinoma. PubMed
Carcinoma, HepatocellularRegulation of glucocorticoids of arginase and argininosuccinate synthetase in cultured rat hepatoma cells. PubMed
Carcinoma, HepatocellularRenal cell carcinoma does not express argininosuccinate synthetase and is highly sensitive to arginine deprivation via arginine deiminase. PubMed
Carcinoma, HepatocellularTargeted cellular metabolism for cancer chemotherapy with recombinant arginine-degrading enzymes. PubMed
Carcinoma, Renal CellRenal cell carcinoma does not express argininosuccinate synthetase and is highly sensitive to arginine deprivation via arginine deiminase. PubMed
CataractUrea cycle enzymes in retina, ciliary body-iris, lens and senile cataracts. PubMed
CitrullinemiaA case of citrullinemia with abnormal messenger RNA for argininosuccinate synthetase. PubMed
CitrullinemiaA GC/MS-based metabolomic approach for diagnosing citrin deficiency. PubMed
CitrullinemiaA novel inborn error of metabolism detected by elevated methionine and/or galactose in newborn screening: neonatal intrahepatic cholestasis caused by citrin deficiency. PubMed
CitrullinemiaA patient with type II citrullinemia who developed refractory complex seizure. PubMed
CitrullinemiaA search for the primary abnormality in adult-onset type II citrullinemia. PubMed
CitrullinemiaAdditional mutations in argininosuccinate synthetase causing citrullinemia. PubMed
CitrullinemiaAn adult-onset case of argininosuccinate synthetase deficiency presenting with atypical citrullinemia. PubMed
CitrullinemiaAnalysis of a splice acceptor site mutation which produces multiple splicing abnormalities in the human argininosuccinate synthetase locus. PubMed
CitrullinemiaAnalysis of deletions at the human argininosuccinate synthetase locus. PubMed
CitrullinemiaApplication of mutation analysis for the previously uncertain cases of adult-onset type II citrullinemia (CTLN2) and their clinical profiles. PubMed
CitrullinemiaArgininosuccinate synthetase activity in cultured human lymphocytes. PubMed
CitrullinemiaArgininosuccinate synthetase deficiency and reye syndrome-like presentation. PubMed
CitrullinemiaArgininosuccinate synthetase deficiency: mutation analysis in 3 Thai patients. PubMed
CitrullinemiaArgininosuccinic acid synthetase deficiency in a hamster cell line and its complementation of argininosuccinic aciduria human fibroblasts. PubMed
CitrullinemiaCharacterization of late-onset citrullinemia 1 in a Korean patient: confirmation by argininosuccinate synthetase gene mutation analysis. PubMed
CitrullinemiaCitrulline and Ammonia Accumulating in Citrullinemia Reduces Antioxidant Capacity of Rat Brain In Vitro. PubMed
CitrullinemiaCitrulline and ammonia accumulating in citrullinemia reduces antioxidant capacity of rat brain in vitro. PubMed
CitrullinemiaCitrullinemia presenting as uncontrollable epilepsy. PubMed
CitrullinemiaCitrullinemia Type I, Classical Variant. Identification of ASS approximately p.G390R (c.1168G>A) mutation in families of a limited geographic area of Argentina: A possible population cluster. PubMed
CitrullinemiaCitrullinemia, argininosuccinate synthetase deficiency. Repository identification No. GM-1044. PubMed
CitrullinemiaCitrullinemia: enzymatic evidence for genetic heterogeneity. PubMed
CitrullinemiaCitrullinemia: quantitative deficiency of argininosuccinate synthetase in the liver. PubMed
CitrullinemiaClearance of amino acids by hemodialysis in argininosuccinate synthetase deficiency. PubMed
CitrullinemiaClinical and biochemical characteristics of patients with urea cycle disorders in a developing country. PubMed
CitrullinemiaComplete neurological recovery of an adult patient with type II citrullinemia after living related partial liver transplantation. PubMed
CitrullinemiaCorrection of argininosuccinate synthetase (AS) deficiency in a murine model of citrullinemia with recombinant adenovirus carrying human AS cDNA. PubMed
CitrullinemiaCT findings in the infantile form of citrullinemia. PubMed
CitrullinemiaDetection of kinetically abnormal argininosuccinate synthase in neonatal citrullinemia by conversion of citrulline to arginine in intact fibroblasts. PubMed
CitrullinemiaDiagnosis and monitoring of inborn errors of metabolism using urease-pretreatment of urine, isotope dilution, and gas chromatography-mass spectrometry. PubMed
CitrullinemiaEffects of glucagon on urinary excretion of urea and on plasma ammonia level in argininosuccinate synthetase deficiency. PubMed
CitrullinemiaElevated plasma concentrations of the endogenous nitric oxide synthase inhibitor asymmetric dimethylarginine in citrullinemia. PubMed
CitrullinemiaGeneration of a mouse model for citrullinemia by targeted disruption of the argininosuccinate synthetase gene. PubMed
CitrullinemiaGenetic approach to prenatal diagnosis in urea cycle defects. PubMed
CitrullinemiaGenomic structure of the adult-onset type II citrullinemia gene, SLC25A13, and cloning and expression of its mouse homologue. PubMed
CitrullinemiaHaemopoietic chimaerism: a complication in heterozygote detection tests for inherited defects in cattle. PubMed
CitrullinemiaHeadache and neuropsychic disorders in the puerperium: a case report with suspected deficiency of urea cycle enzymes. PubMed
CitrullinemiaHepatocellular carcinoma in a case of adult-onset type II citrullinemia. PubMed
CitrullinemiaHepatocyte gene therapy in a large animal: a neonatal bovine model of citrullinemia. PubMed
CitrullinemiaHeterogeneity of mutations in argininosuccinate synthetase causing human citrullinemia. PubMed
CitrullinemiaIdentification of 16 novel mutations in the argininosuccinate synthetase gene and genotype-phenotype correlation in 38 classical citrullinemia patients. PubMed
CitrullinemiaIdentification of two novel mutations in the SLC25A13 gene and detection of seven mutations in 102 patients with adult-onset type II citrullinemia. PubMed
CitrullinemiaImproving the prenatal diagnosis of citrullinemia using citrulline/ornithine+arginine ratio in amniotic fluid. PubMed
CitrullinemiaIn vivo urea cycle flux distinguishes and correlates with phenotypic severity in disorders of the urea cycle. PubMed
CitrullinemiaIncidence and distribution of argininosuccinate synthetase deficiency in human cancers: a method for identifying cancers sensitive to arginine deprivation. PubMed
CitrullinemiaIncreased urinary excretion of argininosuccinate in type II citrullinemia. PubMed
CitrullinemiaInfantile cholestatic jaundice associated with adult-onset type II citrullinemia. PubMed
CitrullinemiaInvestigation of citrullinemia type I variants by in vitro expression studies. PubMed
CitrullinemiaLetter: The high levels of lysine, homocitrulline, and homoarginine found in argininosuccinate synthetase deficiency. PubMed
CitrullinemiaLetter: The presence of the homoanalogues of substrates of the urea cycle in the presence of argininosuccinate synthetase deficiency. PubMed
CitrullinemiaLevel of translatable messenger RNA coding for argininosuccinate synthetase in the liver of the patients with quantitative-type citrullinemia. PubMed
CitrullinemiaLiver transplantation as treatment for neurological disorders. PubMed
CitrullinemiaLiving-related liver transplantation for type II citrullinemia using a graft from heterozygote donor. PubMed
CitrullinemiaLong-term survival of patients with argininosuccinate synthetase deficiency. PubMed
CitrullinemiaMessenger RNA coding for argininosuccinate synthetase in citrullinemia. PubMed
CitrullinemiaMetabolic derangements in deficiency of citrin, a liver-type mitochondrial aspartate-glutamate carrier. PubMed
CitrullinemiaMild citrullinemia in Caucasians is an allelic variant of argininosuccinate synthetase deficiency (citrullinemia type 1). PubMed
CitrullinemiaMolecular analysis of argininosuccinate synthetase deficiency in human fibroblasts. PubMed
CitrullinemiaMolecular definition of bovine argininosuccinate synthetase deficiency. PubMed
CitrullinemiaMRI in a case of adult-onset citrullinemia. PubMed
CitrullinemiaMutation analysis of Korean patients with citrullinemia. PubMed
CitrullinemiaMutations and DNA diagnoses of classical citrullinemia. PubMed
CitrullinemiaMutations and polymorphisms in the human argininosuccinate synthetase (ASS1) gene. PubMed
CitrullinemiaMutations in argininosuccinate synthetase mRNA in Japanese patients, causing classical citrullinemia. PubMed
CitrullinemiaMutations in argininosuccinate synthetase mRNA of Japanese patients, causing classical citrullinemia. PubMed
CitrullinemiaNature and frequency of mutations in the argininosuccinate synthetase gene that cause classical citrullinemia. PubMed
CitrullinemiaNeonatal presentation of adult-onset type II citrullinemia. PubMed
CitrullinemiaNeonatal type of argininosuccinate synthetase deficiency. Report of two cases with autopsy findings. PubMed
CitrullinemiaOrthotopic liver transplantation for urea cycle enzyme deficiency. PubMed
CitrullinemiaPancreatic secretory trypsin inhibitor as a diagnostic marker for adult-onset type II citrullinemia. PubMed
CitrullinemiaPancreatic secretory trypsin inhibitor gene is highly expressed in the liver of adult-onset type II citrullinemia. PubMed
CitrullinemiaPathogenesis and pathophysiology of citrin (a mitochondrial aspartate glutamate carrier) deficiency. PubMed
CitrullinemiaPerinatal pathology casebook. PubMed
CitrullinemiaPlasma concentrations and renal clearance of orotic acid in argininosuccinic acid synthetase deficiency. PubMed
CitrullinemiaPostpartum 'psychosis' in mild argininosuccinate synthetase deficiency. PubMed
CitrullinemiaPregnancy in a healthy woman with untreated citrullinemia. PubMed
CitrullinemiaPrenatal diagnosis of citrullinemia and argininosuccinic aciduria: evidence for a transmission ratio distortion in citrullinemia. PubMed
CitrullinemiaProspective management of a child with neonatal citrullinemia. PubMed
CitrullinemiaProspective treatment of urea cycle disorders. PubMed
CitrullinemiaQualitative abnormality of liver argininosuccinate synthetase in a patient with citrullinemia. PubMed
CitrullinemiaQualitative and quantitative abnormalities of argininosuccinate synthetase in citrullinemia. PubMed
CitrullinemiaRecurrent liver failure in a 25-year-old female. PubMed
CitrullinemiaRetroviral-mediated gene therapy for the treatment of citrullinemia. Transfer and expression of argininosuccinate synthetase in human hematopoietic cells. PubMed
CitrullinemiaSlc25a13-knockout mice harbor metabolic deficits but fail to display hallmarks of adult-onset type II citrullinemia. PubMed
CitrullinemiaStructure of an abnormal messenger RNA for argininosuccinate synthetase in citrullinemia. PubMed
CitrullinemiaStructure of the human argininosuccinate synthetase gene and an improved system for molecular diagnostics in patients with classical and mild citrullinemia. PubMed
CitrullinemiaStudies on complementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts. PubMed
CitrullinemiaStudies on liver argininosuccinate synthetase in a patient with citrullinemia and in normal subjects. PubMed
CitrullinemiaThe heterogeneous distribution of argininosuccinate synthetase in the liver of type II citrullinemic patients. Its specificity and possible clinical implications. PubMed
CitrullinemiaThe human argininosuccinate synthetase locus and citrullinemia. PubMed
CitrullinemiaToxoplasma gondii lacks the enzymes required for de novo arginine biosynthesis and arginine starvation triggers cyst formation. PubMed
CitrullinemiaTransiently reduced activity of carbamyl phosphate synthetase and ornithine transcarbamylase in liver of children with Reye's syndrome. PubMed
CitrullinemiaTwo hypomorphic alleles of mouse Ass1 as a new animal model of citrullinemia type I and other hyperammonemic syndromes. PubMed
CitrullinemiaType II (adult onset) citrullinaemia: clinical pictures and the therapeutic effect of liver transplantation. PubMed
CitrullinemiaType II citrullinemia associated with neutropenia. PubMed
CitrullinemiaType II citrullinemia in an elderly patient treated with living related partial liver transplantation. PubMed
CitrullinemiaUnrecognized citrullinemia mimicking encephalitis in a 14-year-old boy: unexpected result through the use of a standardized lumbar puncture protocol. PubMed
CitrullinemiaUrea cycle disorders in Thai infants: a report of 5 cases. PubMed
CitrullinemiaUsage of cryptic splice sites in citrullinemia fibroblasts suggests role of polyadenylation in splice-site selection during terminal exon definition. PubMed
CitrullinemiaVariation in argininosuccinate synthetase activity in amniotic fluid cell cultures: implications for prenatal diagnosis of citrullinemia. PubMed
Citrullinemia[A case of citrullinemia with cluster type distribution of argininosuccinate synthetase in the liver] PubMed
Citrullinemia[Adult-onset citrullinemia] PubMed,  PubMed
Citrullinemia[Adult-onset type II citrullinemia in a patient undergoing continuous ambulatory peritoneal dialysis] PubMed
Citrullinemia[Argininosuccinate synthetase deficiency] PubMed
Citrullinemia[Recent advances of the treatment in metabolic disorders] PubMed
Citrullinemia[Severe fulminant form of neonatal citrullinemia. Report of a case] PubMed
Citrullinemia[The correlation of consciousness level and the concentration of CSF ammonia in a patient with adult-type citrullinemia] PubMed
ComaLiver transplantation for citrullinaemia improves intellectual function. PubMed
ComaProspective treatment of urea cycle disorders. PubMed
Costello SyndromeDecreased elastin deposition and high proliferation of fibroblasts from Costello syndrome are related to functional deficiency in the 67-kD elastin-binding protein. PubMed
Costello SyndromeThe hyperthermia-enhanced association between tropoelastin and its 67-KDA chaperone results in better deposition of elastic fibers. PubMed
DementiaInducible nitric oxide synthase and argininosuccinate synthetase: co-induction in brain tissue of patients with Alzheimer's dementia and following stimulation with beta-amyloid 1-42 in vitro. PubMed
DystoniaDystonia gene in Ashkenazi Jewish population is located on chromosome 9q32-34. PubMed
DystoniaIdentification of a highly polymorphic microsatellite VNTR within the argininosuccinate synthetase locus: exclusion of the dystonia gene on 9q32-34 as the cause of dopa-responsive dystonia in a large kindred. PubMed
Dystonia Musculorum DeformansLinkage analysis in British and French families with idiopathic torsion dystonia. PubMed
Dystonia Musculorum DeformansLinkage analysis with chromosome 9 markers in hereditary essential tremor. PubMed
EpilepsyDecreased glutamine synthetase, increased citrulline-nitric oxide cycle activities, and oxidative stress in different regions of brain in epilepsy rat model. PubMed
Fatty LiverLong-chain fatty acids suppress the induction of urea cycle enzyme genes by glucocorticoid action. PubMed
Fatty LiverMetabolic derangements in deficiency of citrin, a liver-type mitochondrial aspartate-glutamate carrier. PubMed
FibrosarcomaCumulative influence of elastin peptides and plasminogen on matrix metalloproteinase activation and type I collagen invasion by HT-1080 fibrosarcoma cells. PubMed
fructose-bisphosphatase deficiencyInherited metabolic disorders in Thailand. PubMed
GliomaPresence of argininosuccinate synthetase in glial cells as revealed by peptide-specific antisera. PubMed
Hepatic EncephalopathyLiver transplantation as treatment for neurological disorders. PubMed
Hepatic Encephalopathy[Anesthetic management for a patient with citrullinemia and liver cirrhosis] PubMed
Hepatitis C, Chronic[Serum argininosuccinate synthetase (ASS) in patients with chronic hepatitis C treated with interferon] PubMed
HomocystinuriaInherited metabolic disorders in Thailand. PubMed
HyperargininemiaGenetic approach to prenatal diagnosis in urea cycle defects. PubMed
Hyperglycinemia, NonketoticInherited metabolic disorders in Thailand. PubMed
HyperlysinemiasPeriodic hyperammonemia, hyperlysinemia, and homocitrullinuria associated with decreased argininosuccinate synthetase and arginase activities. PubMed
InfectionSustained generation of nitric oxide and control of mycobacterial infection requires argininosuccinate synthase 1. PubMed
InsulinomaCitrulline-argininosuccinate-arginine cycle coupled to Ca2+-signaling in rat pancreatic beta-cells. PubMed
Kidney Failure, ChronicEffect of chronic renal failure on arginase and argininosuccinate synthetase expression. PubMed
LeukemiaArgininosuccinate synthetase gene expression in leukemias: potential diagnostic marker for blastic crisis of chronic myelocytic leukemia. PubMed
LeukemiaElevated argininosuccinate synthetase activity in adult T leukemia cell lines. PubMed
Leukemia, Myelogenous, Chronic, BCR-ABL PositiveArgininosuccinate synthetase gene expression in leukemias: potential diagnostic marker for blastic crisis of chronic myelocytic leukemia. PubMed
Liver DiseasesClearance of argininosuccinate synthetase from the circulation in acute liver disease. PubMed
Liver DiseasesType II citrullinaemia (citrin deficiency) in a neonate with hypergalactosaemia detected by mass screening. PubMed
Liver Failure, AcuteA pregnant patient with fulminant hepatic failure was found to carry a novel missense mutation in the argininosuccinate synthetase gene. PubMed
Lung NeoplasmsArginine deiminase PEG20 inhibits growth of small cell lung cancers lacking expression of argininosuccinate synthetase. PubMed
Lupus Erythematosus, SystemicElevated gene expression of argininosuccinate synthetase in peripheral lymphocytes from systemic lupus erythematosus (SLE) patients. PubMed
Marfan SyndromeInduction of macrophage chemotaxis by aortic extracts from patients with Marfan syndrome is related to elastin binding protein. PubMed
MelanomaActivation of Ras/PI3K/ERK pathway induces c-Myc stabilization to upregulate argininosuccinate synthetase, leading to arginine deiminase resistance in melanoma cells. PubMed
MelanomaArginine deprivation, autophagy, apoptosis (AAA) for the treatment of melanoma. PubMed
MelanomaArginine deprivation, growth inhibition and tumour cell death: 3. Deficient utilisation of citrulline by malignant cells. PubMed
MelanomaDeprivation of arginine by recombinant human arginase in prostate cancer cells. PubMed
MelanomaElastin-derived peptides upregulate matrix metalloproteinase-2-mediated melanoma cell invasion through elastin-binding protein. PubMed
MelanomaHigh sensitivity of human melanoma cell lines to the growth inhibitory activity of mycoplasmal arginine deiminase in vitro. PubMed
MelanomaNegative argininosuccinate synthetase expression in melanoma tumours may predict clinical benefit from arginine-depleting therapy with pegylated arginine deiminase. PubMed
MelanomaPegylated arginine deiminase (ADI-SS PEG20,000 mw) inhibits human melanomas and hepatocellular carcinomas in vitro and in vivo. PubMed
MelanomaRenal cell carcinoma does not express argininosuccinate synthetase and is highly sensitive to arginine deprivation via arginine deiminase. PubMed
MelanomaResistance to arginine deiminase treatment in melanoma cells is associated with induced argininosuccinate synthetase expression involving c-Myc/HIF-1alpha/Sp4. PubMed
MelanomaTargeted cellular metabolism for cancer chemotherapy with recombinant arginine-degrading enzymes. PubMed
MelanomaThe relationship of arginine deprivation, argininosuccinate synthetase and cell death in melanoma. PubMed
Menkes Kinky Hair SyndromeInherited metabolic disorders in Thailand. PubMed
MesotheliomaIn vivo loss of expression of argininosuccinate synthetase in malignant pleural mesothelioma is a biomarker for susceptibility to arginine depletion. PubMed
MucopolysaccharidosesInherited metabolic disorders in Thailand. PubMed
Mucopolysaccharidosis IImpaired elastogenesis in Hurler disease: dermatan sulfate accumulation linked to deficiency in elastin-binding protein and elastic fiber assembly. PubMed
Neoplasm MetastasisCombined lysosomal protein transmembrane 4 beta-35 and argininosuccinate synthetase expression predicts clinical outcome in hepatocellular carcinoma patients. PubMed
Neoplasm MetastasisReduced argininosuccinate synthetase is a predictive biomarker for the development of pulmonary metastasis in patients with osteosarcoma. PubMed
NeoplasmsA cohort of supporting metabolic enzymes is coinduced with nitric oxide synthase in human tumor cell lines. PubMed
NeoplasmsA proposed interaction mechanism between elastin-derived peptides and the elastin/laminin receptor-binding domain. PubMed
NeoplasmsAberrant regulation of argininosuccinate synthetase by TNF-alpha in human epithelial ovarian cancer. PubMed
NeoplasmsActivation of Ras/PI3K/ERK pathway induces c-Myc stabilization to upregulate argininosuccinate synthetase, leading to arginine deiminase resistance in melanoma cells. PubMed
NeoplasmsADI, autophagy and apoptosis: metabolic stress as a therapeutic option for prostate cancer. PubMed
NeoplasmsArginine deiminase PEG20 inhibits growth of small cell lung cancers lacking expression of argininosuccinate synthetase. PubMed
NeoplasmsArginine deprivation and argininosuccinate synthetase expression in the treatment of cancer. PubMed
NeoplasmsArginine deprivation as a targeted therapy for cancer. PubMed
NeoplasmsArginine deprivation, growth inhibition and tumour cell death: 3. Deficient utilisation of citrulline by malignant cells. PubMed
NeoplasmsCharacterization of chromosome 9 deletions in transitional cell carcinoma by microsatellite assay. PubMed
NeoplasmsCombined lysosomal protein transmembrane 4 beta-35 and argininosuccinate synthetase expression predicts clinical outcome in hepatocellular carcinoma patients. PubMed
NeoplasmsCytotoxicity of human recombinant arginase I (Co)-PEG5000 in the presence of supplemental L-citrulline is dependent on decreased argininosuccinate synthetase expression in human cells. PubMed
NeoplasmsDeprivation of arginine by recombinant human arginase in prostate cancer cells. PubMed
NeoplasmsEngineering an arginine catabolizing bioconjugate: Biochemical and pharmacological characterization of PEGylated derivatives of arginine deiminase from Mycoplasma arthritidis. PubMed
NeoplasmsIncidence and distribution of argininosuccinate synthetase deficiency in human cancers: a method for identifying cancers sensitive to arginine deprivation. PubMed
NeoplasmsInduction of citrulline-nitric oxide (NO) cycle enzymes and NO production in immunostimulated rat RPE-J cells. PubMed
NeoplasmsInduction of inducible nitric oxide synthase, argininosuccinate synthase, and GTP cyclohydrolase I in arthritic joints of human tumor necrosis factor-alpha transgenic mice. PubMed
NeoplasmsMolecular analysis of a myxoid chondrosarcoma with rearrangements of chromosomes 10 and 22. PubMed
NeoplasmsNegative argininosuccinate synthetase expression in melanoma tumours may predict clinical benefit from arginine-depleting therapy with pegylated arginine deiminase. PubMed
NeoplasmsPegylated arginine deiminase (ADI-SS PEG20,000 mw) inhibits human melanomas and hepatocellular carcinomas in vitro and in vivo. PubMed
NeoplasmsPegylated arginine deiminase: a novel anticancer enzyme agent. PubMed
NeoplasmsPhase I/II study of pegylated arginine deiminase (ADI-PEG 20) in patients with advanced melanoma. PubMed
NeoplasmsRecombinant human arginase inhibits proliferation of human hepatocellular carcinoma by inducing cell cycle arrest. PubMed
NeoplasmsRNA interference of argininosuccinate synthetase restores sensitivity to recombinant arginine deiminase (rADI) in resistant cancer cells. PubMed
NeoplasmsTargeted cellular metabolism for cancer chemotherapy with recombinant arginine-degrading enzymes. PubMed
NeoplasmsTumor necrosis factor-alpha reduces argininosuccinate synthase expression and nitric oxide production in aortic endothelial cells. PubMed
Neurologic ManifestationsLiver transplantation as treatment for neurological disorders. PubMed
ornithine carbamoyltransferase deficiencyGenetic approach to prenatal diagnosis in urea cycle defects. PubMed
ornithine carbamoyltransferase deficiencyNew insights in nutritional management and amino acid supplementation in urea cycle disorders. PubMed
Ornithine Carbamoyltransferase Deficiency DiseaseGenetic approach to prenatal diagnosis in urea cycle defects. PubMed
Ornithine Carbamoyltransferase Deficiency DiseaseNew insights in nutritional management and amino acid supplementation in urea cycle disorders. PubMed
OsteosarcomaReduced argininosuccinate synthetase is a predictive biomarker for the development of pulmonary metastasis in patients with osteosarcoma. PubMed
Ovarian NeoplasmsAberrant regulation of argininosuccinate synthetase by TNF-alpha in human epithelial ovarian cancer. PubMed
Ovarian NeoplasmsEpigenetic silencing of argininosuccinate synthetase confers resistance to platinum-induced cell death but collateral sensitivity to arginine auxotrophy in ovarian cancer. PubMed
Pancreatic NeoplasmsPancreatic cancer cell lines deficient in argininosuccinate synthetase are sensitive to arginine deprivation by arginine deiminase. PubMed
PhenylketonuriasInherited metabolic disorders in Thailand. PubMed
PiebaldismSecond locus for Hirschsprung disease/Waardenburg syndrome in a large Mennonite kindred. PubMed
Propionic AcidemiaInherited metabolic disorders in Thailand. PubMed
Prostatic NeoplasmsDeprivation of arginine by recombinant human arginase in prostate cancer cells. PubMed
RetinoblastomaAnti-tumor activity of arginine deiminase via arginine deprivation in retinoblastoma. PubMed
Sarcoma, AvianExpression of human argininosuccinate synthetase after retroviral-mediated gene transfer. PubMed
SepsisHepatic glutamine metabolism in the septic rat. PubMed
StarvationMetabolite regulation of argininosuccinate synthetase in cultured human cells. PubMed
TremorLinkage analysis with chromosome 9 markers in hereditary essential tremor. PubMed
Urea Cycle Disorders, InbornClinical Outcomes of Neonatal Onset Proximal versus Distal Urea Cycle Disorders Do Not Differ. PubMed
Urea Cycle Disorders, InbornNew insights in nutritional management and amino acid supplementation in urea cycle disorders. PubMed
Urea Cycle Disorders, InbornTwo hypomorphic alleles of mouse Ass1 as a new animal model of citrullinemia type I and other hyperammonemic syndromes. PubMed
Urea Cycle Disorders, InbornUnrecognized citrullinemia mimicking encephalitis in a 14-year-old boy: unexpected result through the use of a standardized lumbar puncture protocol. PubMed
Whooping CoughConformational dependence of collagenase (matrix metalloproteinase-1) up-regulation by elastin peptides in cultured fibroblasts. PubMed

REF. AUTHORS TITLE JOURNAL VOL. PAGES YEAR ORGANISMLINK TO PUBMEDSOURCE
1573Shargool, P.D.Purification of argininosuccinate synthetase from cotyledons of germinating peasPhytochemistry102029-20321971Pisum sativum-
1574Ratner, S.Enzymes of arginine and urea synthesisAdv. Enzymol. Relat. Areas Mol. Biol.391-901973Bos taurus PubMed
1575Saheki, T.; Kusumi, T.; Takada, S.; Katsunuma, T.; Katunuma, N.Crystallization and some properties of argininosuccinate synthetase from rat liverFEBS Lett.58314-3171975Rattus norvegicus PubMed
1576Saheki, T.; Kusumi, T.; Takada, S.; Katsunuma, T.Studies of rat liver argininosuccinate synthetase. I. Physicochemical, catalytic, and immunochemical propertiesJ. Biochem.81687-6961977Rattus norvegicus PubMed
1577Rochovansky, O.; Kodowaki, H.; Ratner, S.Biosynthesis of urea. Molecular and regulatory properties of crystalline argininosuccinate synthetaseJ. Biol. Chem.2525287-52941977Bos taurus PubMed
1578Matsuda, Y.; Tsuji, A.; Katunuma, N.; Hayashi, M.; Takahashi, Y.Studies on liver argininosuccinate synthetase in a patient with citrullinemia and in normal subjectsJ. Biochem.85191-1951979Homo sapiens PubMed
1579Hilger, F.; Simon, J.P.; Stalon, V.Yeast argininosuccinate synthetase. Purification, structural and kinetic propertiesEur. J. Biochem.94153-1631979Saccharomyces cerevisiae PubMed
1580Takada, S.; Saheki, T.; Igarashi, Y.; Katsunuma, T.Studies on rat liver argininosuccinate synthetase. Inhibition by various amino acidsJ. Biochem.851309-13141979Rattus norvegicus PubMed
1581O'Brien, W.E.Isolation and characterization of argininosuccinate synthetase from human liverBiochemistry185353-53561979Homo sapiens PubMed
1582Takada, S.; Kusumi, T.; Saheki, T.; Tsuda, M.; Katsunuma, T.Studies of rat liver argininosuccinate synthetase. The presence of three forms, and their physicochemical, catalytic, and immunochemical propertiesJ. Biochem.861353-13591979Rattus norvegicus PubMed
1583Kimball, M.E.; Jacoby, L.B.Purification and properties of argininosuccinate synthetase from normal and canavanine-resistant human lymphoblastsBiochemistry19705-7091980Homo sapiens PubMed
1584Saheki, T.; Tsuda, M.; Takada, S.; Kusumi, K.; Katsunuma, T.Role of argininosuccinate synthetase in the regulation of urea synthesis in the rat and argininosuccinate synthetase-associated metabolic disorder in manAdv. Enzyme Regul.18221-2381986Homo sapiens, Rattus norvegicus-
1585Ratner, S.Argininosuccinate synthetase of bovine liver: chemical and physical propertiesProc. Natl. Acad. Sci. USA795197-51991982Bos taurus PubMed
1586Saheki, T.; Sase, M.; Nakano, K.; Azuma, F.; Katsunuma, T.Some properties of argininosuccinate synthetase purified from human liver and a comparison with the rat liver enzymeJ. Biochem.931531-15371983Homo sapiens, Rattus norvegicus PubMed
1587Raushell, F.M.; Seiglie, J.L.Kinetic mechanism of argininosuccinate synthetaseArch. Biochem. Biophys.225979-9851983Bos taurus PubMed
1588Raushel, F.M.Nitro analogs of substrates for argininosuccinate synthetase and argininosuccinate lyaseArch. Biochem. Biophys.232520-5251984Bos taurus PubMed
1589Kumar, S.; Lennane, J.; Ratner, S.Argininosuccinate synthetase: essential role of cysteine and arginine residues in relation to structure and mechanism of ATP activationProc. Natl. Acad. Sci. USA826745-67491985Bos taurus PubMed
1590Isayama, H.; Nakamura, H.; Kanemura, H.; Kobayashi, K.; Emson, P.C.; Kawabuchi, M.; Tashiro, N.Distribution and co-localization of nitric oxide synthetase and argininosuccinate synthetase in the cat hypothalamusArch. Histol. Cytol.60477-4921997Felis catus PubMed
1591Demarquoy, J.; Fairand, A.; Gautier, C.; Vaillant, T.Regulation of argininosuccinate synthetase level by corticosteroid and pancreatic hormones during perinatal periodMol. Cell. Biochem.14347-511995Rattus norvegicus PubMed
1592Demarquoy, J.; Fairand, A.; Gautier, C.; Vaillant, R.Demonstration of argininosuccinate synthetase activity associated with mitochondrial membrane: characterization and hormonal regulationMol. Cell. Biochem.136145-1551994Rattus norvegicus PubMed
1593Patejunas, G.; Bradley, A.; Beaudet, A.L.; O'Brien, W.E.Generation of a mouse model for citrullinemia by targeted disruption of the argininosuccinate synthetase geneSomatic Cell Mol. Genet.2055-601994Mus musculus PubMed
1594Arnt-Ramos, L.R.; O'Brien, W.E.; Vincent, S.R.Immunohistochemical localization of argininosuccinate synthetase in the rat brain in relation to nitric oxide synthase-containg neuronsNeuroscience51773-7891992Rattus norvegicus PubMed
1595Isahiki, Y.; Noda, T.; Kobayashi, K.; Sase, M.; Saheki, T.; Titani, K.Identification of essential arginine residue(s) for Mg-ATP binding of human argininosuccinate synthetaseProtein Seq. Data Anal.2283-2871989Homo sapiens PubMed
1596Wakui, H.; Komatsuda, A.; Itoh, H.; Kobayashi, R.; Nakamoto, Y.; Miura, A.B.Renal argininosuccinate synthetase: purification, immunohistochemical localization, and elastin-binding propertyRenal Physiol. Biochem.151-91992Sus scrofa PubMed
1597Yu, Y.; Terada, K.; Nagasaki, A.; Takiguchi, M.; Mori, M.Preparation of recombinant argininosuccinate synthetase and arguininosuccinate lyase: expression of the enzymes in rat tissuesJ. Biochem.117952-9571995Rattus norvegicus PubMed
1598Shaheen, N.; Kobayashi, K.; Terazono, H.; Fukushige, T.; Horiuchi, M.; Saheki, T.Characterization of human wild-type and mutant argininosuccinate synthetase proteins expressed in bacterial cellsEnzyme Protein95251-2641994Homo sapiens PubMed
649104Lemke, C.; Yeung, M.; Howell, P.L.Expression, purification, crystallization and preliminary X-ray analysis of Escherichia coli argininosuccinate synthetaseActa Crystallogr. Sect. D552028-20301999Escherichia coli-
650761Shen, L.J.; Lin, W.C.; Beloussow, K.; Shen, W.C.Resistance to the anti-proliferative activity of recombinant arginine deiminase in cell culture correlates with the endogenous enzyme, argininosuccinate synthetaseCancer Lett.191165-1702003Canis lupus familiaris, Homo sapiens, Mus musculus PubMed
651195Van Geldre, L.A.; Timmermans, J.P.; Lefebvre, R.A.L-citrulline recycling by argininosuccinate synthetase and lyase in rat gastric fundusEur. J. Pharmacol.455149-1602002Rattus norvegicus PubMed
652231Lemke, C.T.; Howell, P.L.Substrate induced conformational changes in argininosuccinate synthetaseJ. Biol. Chem.27713074-130812002Escherichia coli PubMed
652242Goto, M.; Nakajima, Y.; Hirotsu, K.Crystal structure of argininosuccinate synthetase from Thermus thermophilus HB8. Structural basis for the catalytic actionJ. Biol. Chem.27715890-158962002Thermus thermophilus PubMed
652404Goto, M.; Omi, R.; Miyahara, I.; Sugahara, M.; Hirotsu, K.Structures of argininosuccinate synthetase in enzyme-ATP substrates and enzyme-AMP product forms. Stereochemistry of the catalytic reactionJ. Biol. Chem.27822964-229712003Thermus thermophilus PubMed
652976Heneka, M.T.; Wiesinger, H.; Dumitrescu-Ozimek, L.; Riederer, P.; Feinstein, D.L.; Klockgether, T.Neuronal and glial coexpression of argininosuccinate synthetase and inducible nitric oxide synthase in Alzheimer diseaseJ. Neuropathol. Exp. Neurol.60906-9162001Homo sapiens PubMed
653046Hukkanen, M.; Platts, L.A.M.; Haralambous, S.; Ainola, M.; Konttinen, Y.T.; Kollias, G.; Polak, J.M.Induction of inducible nitric oxide synthase, argininosuccinate synthase, and GTP cyclohydrolase I in arthritic joints of human tumor necrosis factor-alpha transgenic miceJ. Rheumatol.30652-6592003Mus musculus PubMed
653186Kawahara, K.; Gotoh, T.; Oyadomari, S.; Kajizono, M.; Kuniyasu, A.; Ohsawa, K.; Imai, Y.; Kohsaka, S.; Nakayama, H.; Mori, M.Co-induction of argininosuccinate synthetase, cationic amino acid transporter-2, and nitric oxide synthase in activated murine microglial cellsMol. Brain Res.90165-1732001Mus musculus, Rattus norvegicus PubMed
653344Hammermann, R.; Bliesener, N.; Mossner, J.; Klasen, S.; Wiesinger, H.; Wessler, I.; Racke, K.Inability of rat alveolar macrophages to recycle L-citrulline to L-arginine despite induction of argininosuccinate synthetase mRNA and protein, and inhibition of nitric oxide synthesis by exogenous L-citrullineNaunyn-Schmiedebergs Arch. Pharmacol.358601-6071998Rattus norvegicus PubMed
661285Brasse-Lagnel, C.; Lavoinne, A.; Fairand, A.; Vavasseur, K.; Husson, A.IL-1beta stimulates argininosuccinate synthetase gene expression through NF-kappaB in Caco-2 cellsBiochimie87403-4092005Homo sapiens PubMed
662205Goodwin, B.L.; Solomonson, L.P.; Eichler, D.C.Argininosuccinate synthase expression is required to maintain nitric oxide production and cell viability in aortic endothelial cellsJ. Biol. Chem.27918353-183602004Bos taurus PubMed
662254Hao, G.; Xie, L.; Gross, S.S.Argininosuccinate synthetase is reversibly inactivated by S-nitrosylation in vitro and in vivoJ. Biol. Chem.27936192-362002004Homo sapiens PubMed
662377Pendleton, L.C.; Goodwin, B.L.; Solomonson, L.P.; Eichler, D.C.Regulation of endothelial argininosuccinate synthase expression and NO production by an upstream open reading frameJ. Biol. Chem.28024252-242602005Bos taurus PubMed
671250Goodwin, B.L.; Pendleton, L.C.; Levy, M.M.; Solomonson, L.P.; Eichler, D.C.Tumor necrosis factor-alpha reduces argininosuccinate synthase expression and nitric oxide production in aortic endothelial cellsAm. J. Physiol. Heart Circ. Physiol.293H1115-H11212007Bos taurus PubMed
672445Brasse-Lagnel, C.; Lavoinne, A.; Fairand, A.; Vavasseur, K.; Deniel, N.; Husson, A.Biphasic effect of IL-1beta on the activity of argininosuccinate synthetase in Caco-2 cells. Involvement of nitric oxide productionBiochimie88607-6122006Homo sapiens PubMed
673486Bizzoco, E.; Vannucchi, M.G.; Faussone-Pellegrini, M.S.Transient ischemia increases neuronal nitric oxide synthase, argininosuccinate synthetase and argininosuccinate lyase co-expression in rat striatal neuronsExp. Neurol.204252-2592007Rattus norvegicus PubMed
674048Szlosarek, P.W.; Grimshaw, M.J.; Wilbanks, G.D.; Hagemann, T.; Wilson, J.L.; Burke, F.; Stamp, G.; Balkwill, F.R.Aberrant regulation of argininosuccinate synthetase by TNF-alpha in human epithelial ovarian cancerInt. J. Cancer1216-112007Homo sapiens PubMed
674383Kim, I.S.; Ki, C.S.; Kim, J.W.; Lee, M.; Jin, D.K.; Lee, S.Y.Characterization of late-onset citrullinemia 1 in a Korean patient: confirmation by argininosuccinate synthetase gene mutation analysisJ. Biochem. Mol. Biol.39400-4052006Homo sapiens PubMed
675096Satoh, M.; Iwahori, T.; Sugawara, N.; Yamazaki, M.Liver argininosuccinate synthase binds to bacterial lipopolysaccharides and lipid A and inactivates their biological activitiesJ. Endotoxin Res.1221-382006Mus musculus PubMed
675606Endo, H.; Ootsuka, S.; Fukuda, S.; Kitade, Y.; Saga, N.Functional complementation of an arginine auxotrophic yeast mutant by an argininosuccinate synthetase from Porphyra yezoensis (Rhodophyta)J. Phycol.421066-10712006Porphyra yezoensis-
676120Wagemaker, M.J.; Eastwood, D.C.; van der Drift, C.; Jetten, M.S.; Burton, K.; Van Griensven, L.J.; Op den Camp, H.J.Argininosuccinate synthetase and argininosuccinate lyase: two ornithine cycle enzymes from Agaricus bisporusMycol. Res.111493-5022007Agaricus bisporus PubMed
676636Cruz, C.; Egsgaard, H.; Trujillo, C.; Ambus, P.; Requena, N.; Martins-Loucao, M.A.; Jakobsen, I.Enzymatic evidence for the key role of arginine in nitrogen translocation by arbuscular mycorrhizal fungiPlant Physiol.144782-7922007Daucus carota, Glomus intraradices PubMed
690234Karlberg, T.; Collins, R.; van den Berg, S.; Flores, A.; Hammarstroem, M.; Hoegbom, M.; Holmberg Schiavone, L.; Uppenberg, J.Structure of human argininosuccinate synthetaseActa Crystallogr. Sect. D64279-2862008Homo sapiens PubMed
690727Goodwin, B.L.; Corbin, K.D.; Pendleton, L.C.; Levy, M.M.; Solomonson, L.P.; Eichler, D.C.Troglitazone up-regulates vascular endothelial argininosuccinate synthaseBiochem. Biophys. Res. Commun.370254-2582008Bos taurus PubMed
690765Corbin, K.D.; Pendleton, L.C.; Solomonson, L.P.; Eichler, D.C.Phosphorylation of argininosuccinate synthase by protein kinase ABiochem. Biophys. Res. Commun.3771042-10462008Bos taurus PubMed
690768Guei, T.R.; Liu, M.C.; Yang, C.P.; Su, T.S.Identification of a liver-specific cAMP response element in the human argininosuccinate synthetase geneBiochem. Biophys. Res. Commun.377257-2612008Homo sapiens PubMed
692607Satoh, M.; Ando, S.; Shinoda, T.; Yamazaki, M.Clearance of bacterial lipopolysaccharides and lipid A by the liver and the role of argininosuccinate synthaseInnate Immun.1451-602008Mus musculus PubMed
692659Bowles, T.L.; Kim, R.; Galante, J.; Parsons, C.M.; Virudachalam, S.; Kung, H.J.; Bold, R.J.Pancreatic cancer cell lines deficient in argininosuccinate synthetase are sensitive to arginine deprivation by arginine deiminaseInt. J. Cancer1231950-19552008Homo sapiens PubMed
693030Zhao, Y.; Zhang, J.; Li, H.; Li, Y.; Ren, J.; Luo, M.; Zheng, X.An NADPH sensor protein (HSCARG) down-regulates nitric oxide synthesis by association with argininosuccinate synthetase and is essential for epithelial cell viabilityJ. Biol. Chem.28311004-110132008Homo sapiens PubMed
703248Larovere, L.E.; Angaroni, C.J.; Antonozzi, S.L.; Bezard, M.B.; Shimohama, M.; de Kremer, R.D.Citrullinemia type I, classical variant. Identification of ASS-p~G390R (c.1168G>A) mutation in families of a limited geographic area of Argentina: a possible population clusterClin. Biochem.421166-11682009Homo sapiens PubMed
703251Ibarra-Gonzalez, I.; Fernandez-Lainez, C.; Vela-Amieva, M.Clinical and biochemical characteristics of patients with urea cycle disorders in a developing countryClin. Biochem.43461-4662010Homo sapiens PubMed
703594El Fadili, K.; Drummelsmith, J.; Roy, G.; Jardim, A.; Ouellette, M.Down regulation of KMP-11 in Leishmania infantum axenic antimony resistant amastigotes as revealed by a proteomic screenExp. Parasitol.12351-572009Leishmania infantum PubMed
703969Engel, K.; Höhne, W.; Häberle, J.Mutations and polymorphisms in the human argininosuccinate synthetase (ASS1) geneHum. Mutat.30300-3072009Homo sapiens PubMed
704070Nicholson, L.J.; Smith, P.R.; Hiller, L.; Szlosarek, P.W.; Kimberley, C.; Sehouli, J.; Koensgen, D.; Mustea, A.; Schmid, P.; Crook, T.Epigenetic silencing of argininosuccinate synthetase confers resistance to platinum-induced cell death but collateral sensitivity to arginine auxotrophy in ovarian cancerInt. J. Cancer1251454-14632009Homo sapiens PubMed
704078Delage, B.; Fennell, D.A.; Nicholson, L.; McNeish, I.; Lemoine, N.R.; Crook, T.; Szlosarek, P.W.Arginine deprivation and argininosuccinate synthetase expression in the treatment of cancerInt. J. Cancer1262762-27722010Homo sapiens PubMed
704509Guerreiro, J.R.; Lameu, C.; Oliveira, E.F.; Klitzke, C.F.; Melo, R.L.; Linares, E.; Augusto, O.; Fox, J.W.; Lebrun, I.; Serrano, S.M.; Camargo, A.C.Argininosuccinate synthetase is a functional target for a snake venom anti-hypertensive peptide: role in arginine and nitric oxide productionJ. Biol. Chem.28420022-200332009Homo sapiens, Mus musculus PubMed
705506Nagasaka, H.; Tsukahara, H.; Yorifuji, T.; Miida, T.; Murayama, K.; Tsuruoka, T.; Takatani, T.; Kanazawa, M.; Kobayashi, K.; Okano, Y.; Takayanagi, M.Evaluation of endogenous nitric oxide synthesis in congenital urea cycle enzyme defectsMetab. Clin. Exp.58278-2822009Homo sapiens PubMed
715188Prima, V.; Wang, A.; Molina, G.; Wang, K.K.; Svetlov, S.I.Inhibition of LPS toxicity by hepatic argininosuccinate synthase (ASS): Novel roles for ASS in innate immune responses to bacterial infectionInt. Immunopharmacol.111180-11882011Homo sapiens, Mus musculus PubMed
715624Mun, G.I.; Kim, I.S.; Lee, B.H.; Boo, Y.C.Endothelial argininosuccinate synthetase 1 regulates nitric oxide production and monocyte adhesion under static and laminar shear stress conditionsJ. Biol. Chem.2862536-25422011Homo sapiens PubMed

LINKS TO OTHER DATABASES (specific for EC-Number 6.3.4.5)
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