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arginine + 3-aminopropionic acid
3-guanidinopropionic acid + ornithine
-
-
-
-
?
arginine + 4-aminobutyric acid
4-guanidinobutyric acid + ornithine
-
-
-
-
?
arginine + delta-aminovaleric acid
delta-guanidinovaleric acid + ornithine
-
-
-
-
?
arginine + ethanolamine
2-guanidinoethanol + ornithine
-
-
-
-
?
arginine + gamma-aminobutyric acid
gamma-guanidinobutyric acid + ornithine
-
-
-
-
?
canavanine + canaline
canaline + canavanine
-
-
-
-
?
canavanine + glycine
guanidinoacetate + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
guanidinoacetate + hydroxylamine
hydroxyguanidine + glycine
-
-
-
-
?
homoarginine + glycine
homoornithine + guanidinoacetate
-
-
-
-
r
hydroxyguanidine + glycine
hydroxylamine + guanidinoacetate
-
-
-
-
?
L-alanine + glycine
?
-
-
-
-
?
L-aminobutyric acid + glycine
?
-
-
-
-
?
L-arginine + glycine
L-ornithine + guanidinoacetate
L-norvaline + glycine
?
-
-
-
-
?
L-ornithine + guanidinoacetate
L-arginine + glycine
additional information
?
-
-
homoarginine, alpha-amino-gamma-guanidinobutyric acid and alpha-amino-beta-guanidinopropionic acid are not substrates
-
-
?
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
r
canavanine + ornithine
arginine + canaline
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
-
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
first reaction in the de novo biosynthesis of creatine
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
first reaction in the de novo biosynthesis of creatine
-
-
r
L-arginine + glycine
L-ornithine + guanidinoacetate
-
enzyme is involved in biosynthesis of creatine. Patients with AGAT deficiency show mental and motor retardation and severe delay in speech development. Both creatine and guanidinoacetate are decreased in body fluids of AGAT-deficient patients
-
-
?
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
r
L-ornithine + guanidinoacetate
L-arginine + glycine
-
-
-
r
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Acute Kidney Injury
Interrelation of urinary and plasma levels of guanidinoacetic acid with alteration in renal activity of glycine amidinotransferase in acute renal failure rats.
Acute Kidney Injury
[Activity of glycine amidinotransferase and its isoforms in dog kidney tissue normally and in acute renal insufficiency]
Breast Neoplasms
Creatine-mediated crosstalk between adipocytes and cancer cells regulates obesity-driven breast cancer.
Carcinoma
A short review on creatine-creatine kinase system in relation to cancer and some experimental results on creatine as adjuvant in cancer therapy.
Carcinoma
Enzymes of creatine biosynthesis, arginine and methionine metabolism in normal and malignant cells.
Dent Disease
Novel Fanconi renotubular syndromes provide insights in proximal tubule pathophysiology.
Diabetes Mellitus
[Study of impaired metabolism of guanidinoacetic acid in uremia--the compensatory role of the pancreas in guanidinoacetic acid synthesis]
Fanconi Syndrome
Glycine Amidinotransferase (GATM), Renal Fanconi Syndrome, and Kidney Failure.
Genetic Diseases, Inborn
LC-MS/MS measurements of urinary guanidinoacetic acid and creatine: Method optimization by deleting derivatization step.
Glomerulonephritis
[Glycine amidinotransferase activity of the blood and urine of children with acute streptococcal glomerulonephritis]
glycine amidinotransferase deficiency
Arginine-Glycine Amidinotransferase Deficiency and Functional Characterization of Missense Variants in GATM.
glycine amidinotransferase deficiency
Arginine:glycine amidinotransferase (AGAT) deficiency in a newborn: early treatment can prevent phenotypic expression of the disease.
glycine amidinotransferase deficiency
Arginine:glycine amidinotransferase deficiency: a treatable metabolic encephalomyopathy.
glycine amidinotransferase deficiency
Arginine:glycine amidinotransferase deficiency: the third inborn error of creatine metabolism in humans.
glycine amidinotransferase deficiency
Cerebral creatine deficiency syndromes: clinical aspects, treatment and pathophysiology.
glycine amidinotransferase deficiency
Creatine and creatine deficiency syndromes: biochemical and clinical aspects.
glycine amidinotransferase deficiency
Creatine metabolism in urea cycle defects.
glycine amidinotransferase deficiency
Developmental progress and creatine restoration upon long-term creatine supplementation of a patient with arginine:glycine amidinotransferase deficiency.
glycine amidinotransferase deficiency
Diagnostic enzyme assay that uses stable-isotope-labeled substrates to detect L-arginine:glycine amidinotransferase deficiency.
glycine amidinotransferase deficiency
Fifteen-year follow-up of Italian families affected by arginine glycine amidinotransferase deficiency.
glycine amidinotransferase deficiency
Homoarginine- and Creatine-Dependent Gene Regulation in Murine Brains with l-Arginine:Glycine Amidinotransferase Deficiency.
glycine amidinotransferase deficiency
Inborn errors of creatine metabolism and epilepsy: clinical features, diagnosis, and treatment.
glycine amidinotransferase deficiency
L-arginine:glycine amidinotransferase deficiency protects from metabolic syndrome.
glycine amidinotransferase deficiency
LC-MS/MS measurements of urinary guanidinoacetic acid and creatine: Method optimization by deleting derivatization step.
guanidinoacetate n-methyltransferase deficiency
Cerebral creatine deficiency syndromes: clinical aspects, treatment and pathophysiology.
guanidinoacetate n-methyltransferase deficiency
Creatine and creatine deficiency syndromes: biochemical and clinical aspects.
guanidinoacetate n-methyltransferase deficiency
Creatine metabolism in urea cycle defects.
guanidinoacetate n-methyltransferase deficiency
Guanidinoacetate and creatine plus creatinine assessment in physiologic fluids: an effective diagnostic tool for the biochemical diagnosis of arginine:glycine amidinotransferase and guanidinoacetate methyltransferase deficiencies.
guanidinoacetate n-methyltransferase deficiency
LC-MS/MS measurements of urinary guanidinoacetic acid and creatine: Method optimization by deleting derivatization step.
Gyrate Atrophy
Inhibition of arginine-glycine amidinotransferase by ornithine. A possible mechanism for the muscular and chorioretinal atrophies in gyrate atrophy of the choroid and retina with hyperornithinemia.
Gyrate Atrophy
Ornithine delta-aminotransferase activity in retina and other tissues.
Heart Failure
Myocardial expression of the arginine:glycine amidinotransferase gene is elevated in heart failure and normalized after recovery: potential implications for local creatine synthesis.
Intellectual Disability
Arginine:glycine amidinotransferase (AGAT) deficiency in a newborn: early treatment can prevent phenotypic expression of the disease.
Intellectual Disability
Creatine depletion in a new case with AGAT deficiency: clinical and genetic study in a large pedigree.
Intellectual Disability
Fifteen-year follow-up of Italian families affected by arginine glycine amidinotransferase deficiency.
Intellectual Disability
Inborn errors of creatine metabolism and epilepsy: clinical features, diagnosis, and treatment.
Kidney Failure, Chronic
Impaired metabolism of guanidinoacetic acid in uremia.
Language Disorders
Inborn errors of creatine metabolism and epilepsy: clinical features, diagnosis, and treatment.
Metabolic Syndrome
Differential regulation of AMPK activation in leptin- and creatine-deficient mice.
Metabolic Syndrome
L-arginine:glycine amidinotransferase deficiency protects from metabolic syndrome.
Metabolism, Inborn Errors
Health implications of creatine: can oral creatine supplementation protect against neurological and atherosclerotic disease?
Metabolism, Inborn Errors
[Cerebral creatine transporter deficiency: an infradiagnosed neurometabolic disease]
Muscular Diseases
Creatine deficiency syndrome. A treatable myopathy due to arginine-glycine amidinotransferase (AGAT) deficiency.
Muscular Diseases
Does reduced creatine synthesis protect against statin myopathy?
Muscular Diseases
Muscle phenotype of AGAT- and GAMT-deficient mice after simvastatin exposure.
Muscular Diseases
Statins and skeletal muscles toxicity: from clinical trials to everyday practice.
Muscular Dystrophies
Reduced renal arginine-glycine transamidinase activity in myotonic goats and in patients with myotonic muscular dystrophy.
Myotonic Dystrophy
Arginine-glycine amidinotransferase activity in myotonic dystrophy.
Neoplasm Metastasis
Creatine promotes cancer metastasis through activation of Smad2/3.
Neoplasms
Creatine-mediated crosstalk between adipocytes and cancer cells regulates obesity-driven breast cancer.
Neoplasms
Enzymes of creatine biosynthesis, arginine and methionine metabolism in normal and malignant cells.
Nephrosis, Lipoid
[Glycine transamidinase (ADT) activity in the serum and urine of children with lipoid nephrosis]
Pancreatitis
[Repression and depression of glycine amidinotransferase in the rat pancreas at different stages of the development of pancreatitis]
Renal Insufficiency
Genetic mitochondrial glycine amidinotransferase protein aggregate formation triggers microparticle sensing and kidney failure.
Renal Insufficiency
Glycine Amidinotransferase (GATM), Renal Fanconi Syndrome, and Kidney Failure.
Renal Insufficiency
Interrelation of urinary and plasma levels of guanidinoacetic acid with alteration in renal activity of glycine amidinotransferase in acute renal failure rats.
Renal Insufficiency
[Study of impaired metabolism of guanidinoacetic acid in uremia--the compensatory role of the pancreas in guanidinoacetic acid synthesis]
Sarcoma
A short review on creatine-creatine kinase system in relation to cancer and some experimental results on creatine as adjuvant in cancer therapy.
Sarcoma
Enzymes of creatine biosynthesis, arginine and methionine metabolism in normal and malignant cells.
Sarcoma 180
A short review on creatine-creatine kinase system in relation to cancer and some experimental results on creatine as adjuvant in cancer therapy.
Sarcoma 180
Enzymes of creatine biosynthesis, arginine and methionine metabolism in normal and malignant cells.
Stroke
Homoarginine Levels Are Regulated by L-Arginine:Glycine Amidinotransferase and Affect Stroke Outcome: Results From Human and Murine Studies.
Stroke
Homoarginine- and Creatine-Dependent Gene Regulation in Murine Brains with l-Arginine:Glycine Amidinotransferase Deficiency.
Tuberous Sclerosis
Proteomes analysis reveals the involvement of autophagy in AD-like neuropathology induced by noise exposure and ApoE4.
Uremia
Impaired metabolism of guanidinoacetic acid in uremia.
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Walker, J.B.
Amidinotranferases
The Enzymes, 3rd Ed. (Boyer, P. D. , ed. )
9
497-509
1973
Bos taurus, Canavalia ensiformis, Gallus gallus, Homo sapiens, Lacerta sp., Rana sp., Rattus norvegicus, Sus scrofa
-
brenda
Dubach, U.C.
Transamidinase
Methods Enzym. Anal. , 3rd Ed. (Bergmeyer, H. U. , ed. )
1
740-743
1974
Homo sapiens, Rattus norvegicus, Sus scrofa
-
brenda
Sipilae, I.
Inhibition of arginine-glycine amidinotransferase by ornithine. A possible mechanism for the muscular and chorioretinal atrophies in gyrate atrophy of the choroid and retina with hyperornithinemia
Biochim. Biophys. Acta
613
79-84
1980
Gallus gallus, Homo sapiens, Rattus norvegicus, Rattus norvegicus Sprague-Dawley, Sus scrofa
brenda
Gross, M.D.; Eggen, M.A.; Simon, A.M.; Van Pilsum, J.F.
The purification and charcterization of human kidney L-arginine:glycine amidinotransferase
Arch. Biochem. Biophys.
251
747-755
1986
Homo sapiens, Rattus norvegicus, Sus scrofa
brenda
Guthmiller, P.; Van Pilsum, J.F.; Boen, J.R.; McGuire, D.M.
Cloning and sequencing of rat kidney L-arginine:glycine amidinotransferase. Studies on the mechanism of regulation by growth hormone and creatine
J. Biol. Chem.
269
17556-17560
1994
Homo sapiens, Rattus norvegicus, Sus scrofa
brenda
Watanabe, Y.; Van Pilsum, J.F.; Yokoi, I.; Mori, A.
Synthesis of neuroactive guanidino compounds by rat kidney L-arginine:glycine amidinotransferase
Life Sci.
55
351-358
1994
Felis domestica, Homo sapiens, Mus musculus, Oryctolagus cuniculus, Rattus norvegicus, Rattus norvegicus Sprague-Dawley
brenda
Humm, A.; Fritsche, E.; Mann, K.; Goehl, M.; Huber, R.
Recombinant expression and isolation of human L-arginine:glycine amidinotransferase and identification of its active-site cysteine residue
Biochem. J.
322
771-776
1997
Homo sapiens, Homo sapiens (P50440), Sus scrofa, Rattus norvegicus (P50442)
-
brenda
Humm, A.; Fritsche, E.; Steinbacher, S.
Structure and reaction mechanism of L-arginine:glycine amidinotransferase
Biol. Chem.
378
193-197
1997
Homo sapiens, Rattus norvegicus, Sus scrofa
brenda
Humm, A.; Fritsche, E.; Steinbacher, S.; Huber, R.
Crystal structure and mechanism of human L-arginine:glycine amidinotransferase: a mitochondrial enzyme involved in creatine biosynthesis
EMBO J.
16
3373-3385
1997
Homo sapiens, Homo sapiens (P50440), Rattus norvegicus, Rattus norvegicus (P50442), Sus scrofa
brenda
Bedekar, A.; Zink, R.M.; Sherman, D.H.; Line, T.V.; Van Pilsum, J.F.
The comparative amino acid sequences, substrate specificities and gene or cDNA nucleotide sequences of some prokaryote and eukaryote amidinotransferases: implications for evolution
Comp. Biochem. Physiol. B
119B
677-690
1998
Homo sapiens, Lampetra planeri, no activity in Streptomyces sp., Rattus norvegicus, Sus scrofa
-
brenda
Fritsche, E.; Humm, A.; Huber, R.
The ligand-induced structural changes of human L-arginine:glycine amidinotransferase. A mutational and crystallographic study
J. Biol. Chem.
274
3026-3032
1999
Homo sapiens
brenda
Zhu, Y.; Evans, M.I.
Estrogen modulates the expression of L-arginine:glycine amidinotransferase in chick liver
Mol. Cell. Biochem.
221
139-145
2001
Gallus gallus, Gallus gallus (Q9I9K9), Homo sapiens, Rana sp., Rattus norvegicus, Sus scrofa
brenda
Carducci, C.; Birarelli, M.; Leuzzi, V.; Carducci, C.; Battini, R.; Cioni, G.; Antonozzi, I.
Guanidinoacetate and creatine plus creatinine assessment in physiologic fluids: an effective diagnostic tool for the biochemical diagnosis of arginine:glycine amidinotransferase and guanidinoacetate methyltransferase deficiencies
Clin. Chem.
48
1772-1778
2002
Homo sapiens
brenda
Verhoeven, N.M.; Schor, D.S.M.; Roos, B.; Battini, R.; Stockler-Ipsiroglu, S.; Salomons, G.S.; Jakobs, C.
Diagnostic enzyme assay that uses stable-isotope-labeled substrates to detect L-arginine: glycine amidinotransferase deficiency
Clin. Chem.
49
803-805
2003
Homo sapiens
brenda
Verhoeven, N.M.; Salomons, G.S.; Jakobs, C.
Laboratory diagnosis of defects of creatine biosynthesis and transport
Clin. Chim. Acta
361
1-9
2005
Homo sapiens
brenda
Choe, C.U.; Atzler, D.; Wild, P.S.; Carter, A.M.; Boeger, R.H.; Ojeda, F.; Simova, O.; Stockebrand, M.; Lackner, K.; Nabuurs, C.; Marescau, B.; Streichert, T.; Mueller, C.; Lueneburg, N.; De Deyn, P.P.; Benndorf, R.A.; Baldus, S.; Gerloff, C.; Blankenberg, S.; Heerschap, A.; Grant, P.J.; Magnus, T.; Zeller, T.; Isbrandt, D.; Schwedhelm, E.
Homoarginine levels are regulated by L-arginine:glycine amidinotransferase and affect stroke outcome: results from human and murine studies
Circulation
128
1451-1461
2013
Homo sapiens (P50440), Homo sapiens, Mus musculus (Q9D964), Mus musculus, Mus musculus C57BL6 (Q9D964)
brenda