Information on EC 3.7.1.2 - fumarylacetoacetase:

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EC NUMBERCOMMENTARY
3.7.1.2-

RECOMMENDED NAMEGeneOntology No.
fumarylacetoacetaseGO:0004334

REACTIONREACTION DIAGRAMCOMMENTARYORGANISM UNIPROT ACCESSION NO.LITERATURE
4-fumarylacetoacetate + H2O = acetoacetate + fumarate
show the reaction diagram
----
4-fumarylacetoacetate + H2O = acetoacetate + fumarate
show the reaction diagram
also acts on other 3,5- and 2,4-dioxo acids, mechanism involves a catalytic metal ion, a Glu/His catalyic diad, and a charged oxyanion holeHomo sapiens-655997
4-fumarylacetoacetate + H2O = acetoacetate + fumarate
show the reaction diagram
also acts on other 3,5- and 2,4-dioxo acids, mechanism involves a catalytic metal ion, a Glu/His catalyic diad, and a charged oxyanion hole, side chain mobility in the active siteMus musculus-655997

REACTION TYPEORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
C-C-bond cleavageHomo sapiens, Mus musculus--655997
hydrolysis of C-C bond----
hydrolysis of C-C bondThermus thermophilus--684183
hydrolysis of C-C bondHomo sapiens, Mus musculus--684964
hydrolysis of C-C bondCaenorhabditis elegansQ94272-687809

PATHWAYKEGG LinkMetaCyc Link
tyrosine degradation I-TYRFUMCAT-PWY

SYSTEMATIC NAMEIUBMB Comments
4-fumarylacetoacetate fumarylhydrolaseAlso acts on other 3,5- and 2,4-dioxo acids.

SYNONYMSORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
4-fumarylacetoacetate fumarylhydrolase----
acylpyruvase----
AtFAHArabidopsis thaliana--670622
beta-diketonase----
beta-diketonaseHomo sapiens, Mus musculus--655997
diketo acid hydrolase----
FAA----
FAAThermus thermophilus--684183
FAA hydrolaseHomo sapiens, Mus musculus--684964
FAH----
FAHMus musculus--655997, 657866, 684964, 697797, 699824
FAHHomo sapiens--655997, 684964
FAHBos taurus--657866
FAHArabidopsis thaliana--670622
FAHRattus norvegicus--670957
FAHPseudomonas chlororaphis--686266
FAHCaenorhabditis elegansQ94272-687809
FAHSulfolobus solfataricus--693645
FAHVitis vinifera x Vitis riparia, Vitis vinifera x Vitis vinifera--706809
fumarylacetoacetaseThermus thermophilus--684183
fumarylacetoacetaseHomo sapiens, Mus musculus--684964
fumarylacetoacetaseCaenorhabditis elegansQ94272-687809
fumarylacetoacetaseVitis vinifera x Vitis riparia, Vitis vinifera x Vitis vinifera--706809
fumarylacetoacetate hydrolase----
fumarylacetoacetate hydrolaseMus musculus--655997, 657866, 684964, 697797, 699824
fumarylacetoacetate hydrolaseHomo sapiens--655997, 684964
fumarylacetoacetate hydrolaseBos taurus--657866
fumarylacetoacetate hydrolasePseudomonas putida--669086
fumarylacetoacetate hydrolaseArabidopsis thaliana--670622
fumarylacetoacetate hydrolaseRattus norvegicus--670957
fumarylacetoacetate hydrolasePseudomonas chlororaphis--686266
fumarylacetoacetate hydrolaseCaenorhabditis elegansQ94272-687809
fumarylacetoacetate hydrolaseSulfolobus solfataricus-C-terminal domain (residues 76-293) of the enzyme 2-keto-3-deoxy-D-arabinonate dehydratase resembles the catalytic domain of members of the fumarylacetoacetate hydrolase protein family693645
HmgBPseudomonas putida--669086
HmgBPseudomonas chlororaphis--686266
mFAHMus musculus--684964
TTHA0809Thermus thermophilus--684183

CAS REGISTRY NUMBERCOMMENTARY
9032-59-1-

ORGANISMCOMMENTARYLITERATURESEQUENCE CODESEQUENCE DB SOURCE
Arabidopsis thaliana-670622--Manually annotated by BRENDA team
Bos taurus-289239, 289240, 289241, 289242, 289243, 289246, 657866--Manually annotated by BRENDA team
Caenorhabditis elegans-687809Q94272UniProtManually annotated by BRENDA team
Felis catus-289238--Manually annotated by BRENDA team
Homo sapiens-289244, 289247, 289248, 289249, 289252, 289253, 289255, 289256, 655997, 684964--Manually annotated by BRENDA team
Mus musculus-289238, 289251, 289254, 289257, 655997, 657866, 699824--Manually annotated by BRENDA team
Mus musculus-684964, 697797P35505UniProtManually annotated by BRENDA team
Oryctolagus cuniculus-289238--Manually annotated by BRENDA team
Pseudomonas chlororaphisstrain O6686266--Manually annotated by BRENDA team
Pseudomonas putidastrain U-dhmgB669086--Manually annotated by BRENDA team
Pseudomonas putida U-dhmgBstrain U-dhmgB669086--Manually annotated by BRENDA team
Rattus norvegicus-289238, 289240, 289245, 289250, 289253, 670957--Manually annotated by BRENDA team
Sulfolobus solfataricus-693645--Manually annotated by BRENDA team
Thermus thermophilus-684183--Manually annotated by BRENDA team
Vitis vinifera x Vitis riparia-706809--Manually annotated by BRENDA team
Vitis vinifera x Vitis vinifera-706809--Manually annotated by BRENDA team

GENERAL INFORMATIONORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
physiological functionVitis vinifera x Vitis riparia, Vitis vinifera x Vitis vinifera-involved in aromatic amino acid metabolism706809

SUBSTRATEPRODUCT                      REACTION DIAGRAMORGANISM UNIPROT ACCESSION NO. COMMENTARY/
Substrate
LITERATURE/
Substrate
COMMENTARY/
Product
LITERATURE/
Product
Reversibility
r=reversible
ir=irreversible
?=not specified
2,4-diketo-4-(4-methylphenyl) butyrate + H2Opyruvate + (4-methylphenyl)acetate
show the reaction diagram
Rattus norvegicus--289238-289238?
2,4-diketo-4-phenyl butyrate + H2Opyruvate + phenylacetate
show the reaction diagram
Rattus norvegicus--289238-289238?
2,4-diketo-5,5-dimethyl hexanoate + H2Opyruvate + 2,2-dimethylpropanoate
show the reaction diagram
Rattus norvegicus--289238-289238?
2,4-diketo-5-methyl heptanoate + H2Opyruvate + 2-methylbutanoate
show the reaction diagram
Rattus norvegicus--289238-289238?
2,4-diketo-5-methyl hexanoate + H2Opyruvate + isobutanoate
show the reaction diagram
Rattus norvegicus--289238-289238?
2,4-diketocaprate + H2Opyruvate + heptanoate
show the reaction diagram
Rattus norvegicus--289238-289238?
2,4-diketoheptanoate + H2Opyruvate + butanoate
show the reaction diagram
Rattus norvegicus--289238-289238?
2,4-diketohexanoate + H2Opyruvate + propanoate
show the reaction diagram
Rattus norvegicus--289238-289238?
2,4-diketohexanoate + H2Opyruvate + propanoate
show the reaction diagram
Bos taurus--289239-289239?
2,4-diketononanoate + H2Opyruvate + hexanoate
show the reaction diagram
Rattus norvegicus--289238-289238?
2,4-diketooctanoate + H2Opyruvate + pentanoate
show the reaction diagram
Rattus norvegicus--289238-289238?
2,4-diketoundecanoate + H2Opyruvate + octanoate
show the reaction diagram
Rattus norvegicus--289238-289238?
2,4-diketovalerate + H2Opyruvate + acetate
show the reaction diagram
Rattus norvegicus--289238-289238?
3,5-dioxohexanoate + H2O?
show the reaction diagram
Bos taurus--289243-289243?
3-benzoylpropionate + H2Obenzoate + propionate
show the reaction diagram
Bos taurus--289242-289242?
3-phenylpropionopyruvate + H2O?
show the reaction diagram
Bos taurus--289243-289243?
4-ethyl oxaloacetate + H2O?
show the reaction diagram
Bos taurus--289242-289242?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Mus musculus--289257-289257?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Mus musculus--657866--?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Mus musculusP35505-684964--?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Thermus thermophilus--684183--?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Homo sapiens--289252---
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Homo sapiens--289247-289247?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Homo sapiens--655997, 684964--?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Rattus norvegicus--289240-289240?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Rattus norvegicus--289245-289245?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Rattus norvegicus--670957--?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Bos taurus--289240-289240?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Bos taurus--289241-289241?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Bos taurus--289242-289242?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Bos taurus--289246-289246?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Bos taurus--657866--?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Arabidopsis thaliana--670622--ir
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Pseudomonas putida--669086--ir
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Caenorhabditis elegansQ94272-687809--?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Mus musculus, Homo sapiens-final step of Phe and Tyr degradation, essential metabolic function, loss of enzyme function causes the fatal metabolic disease hereditary tyrosinemia type I, i.e. HT1655997--?
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Mus musculus-formation of a tetrahedral alkoxy transition state intermediate655997--?
4-ketopimelate + H2Osuccinate + propanoate
show the reaction diagram
Bos taurus--289242-289242?
5-phenyl-3,5-dioxopentanoate + H2O?
show the reaction diagram
Bos taurus--289243-289243?
6,6-dimethyl-3,5-dioxoheptanoate + H2O?
show the reaction diagram
Bos taurus--289243-289243?
acetoacetate + H2Oacetate
show the reaction diagram
Bos taurus--289242-289242?
acetopyruvate + H2O?
show the reaction diagram
Bos taurus--289243-289243?
acetopyruvic acid + H2Oacetic acid + pyruvic acid
show the reaction diagram
Bos taurus--289241-289241?
acetylpyruvate + H2O?
show the reaction diagram
Bos taurus--657866--?
alpha-ketobutyrate + H2O?
show the reaction diagram
Bos taurus--289242-289242?
alpha-ketoglutarate + H2O?
show the reaction diagram
Bos taurus--289242-289242?
benzoylpyruvate + H2Opyruvate + benzoate
show the reaction diagram
Bos taurus--289243-289243?
butyropyruvate + H2O?
show the reaction diagram
Bos taurus--289243-289243?
dihydroxytartrate + H2O?
show the reaction diagram
Bos taurus--289242-289242?
maleylacetoacetate + H2Oacetoacetate + maleate
show the reaction diagram
Arabidopsis thaliana--670622--?
oxaloacetate + H2O?
show the reaction diagram
Bos taurus--289242--?
pivalopyruvate + H2O?
show the reaction diagram
Bos taurus--289243-289243?
propionopyruvate + H2O?
show the reaction diagram
Bos taurus--289242-289242?
propionopyruvate + H2O?
show the reaction diagram
Bos taurus--289243-289243?
propionopyruvate + H2O?
show the reaction diagram
Bos taurus--289246-289246?
pyruvate + H2O?
show the reaction diagram
Bos taurus--289242-289242?
succinylacetoacetate + H2O?
show the reaction diagram
Homo sapiens--289244-289244?
triacetic acid + H2Oacetoacetic acid + acetic acid
show the reaction diagram
Bos taurus--289239-289239?

NATURAL SUBSTRATESNATURAL PRODUCTSREACTION DIAGRAMORGANISM UNIPROT ACCESSION NO.COMMENTARY SUBSTRATELITERATURE
(Substrate)
COMMENTARY PRODUCTLITERATURE
(Product)
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Mus musculus--289257-289257
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Mus musculusP35505-684964--
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Thermus thermophilus--684183--
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Homo sapiens--289247-289247
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Homo sapiens--684964--
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Rattus norvegicus--289240-289240
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Rattus norvegicus--289245-289245
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Bos taurus--289240-289240
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Bos taurus--289241-289241
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Bos taurus--289242-289242
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Bos taurus--289246-289246
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Caenorhabditis elegansQ94272-687809--
4-fumarylacetoacetate + H2Oacetoacetate + fumarate
show the reaction diagram
Mus musculus, Homo sapiens-final step of Phe and Tyr degradation, essential metabolic function, loss of enzyme function causes the fatal metabolic disease hereditary tyrosinemia type I, i.e. HT1655997--
pyruvate + H2O?
show the reaction diagram
Bos taurus--289242-289242

COFACTORORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATUREIMAGE
No entries in this field

METALS and IONS ORGANISM UNIPROT ACCESSION NO.COMMENTARY LITERATURE
Ca2+Mus musculus--289257, 657866
Ca2+Homo sapiens--655997
Ca2+Mus musculus-bound to the free enzyme655997
Cu2+Bos taurus--289239
Mg2+Bos taurus--289239
Mg2+Homo sapiens, Mus musculus-bound to the active site of the enzyme655997
Mg2+Mus musculus-stabilizes the beta-roll that constitutes the active site of the enzyme657866
additional informationMus musculus-metal ligand binding distance analysis from crystal structure655997

INHIBITORSORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE IMAGE
3-benzoylpropionateBos taurus-1 mM, 40% inhibition289242 2D-image
3-[(3-carboxy-2-oxopropyl)hydroxyphosphinyl]acrylateHomo sapiens, Mus musculus-COPHPAA684964 2D-image
4-(hydroxymethylphosphinoyl)-3-oxo-butanoic acidHomo sapiens, Mus musculus-i.e. HMPOBA, competitive655997 2D-image
4-ethyl oxaloacetateBos taurus-1 mM, 30% inhibition289242 2D-image
4-ketopimelateBos taurus-1 mM, 10% inhibition289242 2D-image
4-[(2-carboxyethyl)-hydroxyphosphinyl]-3-oxobutyrateHomo sapiens, Mus musculus-CEHPOBA684964 2D-image
5,5'-dithiobis(2-nitrobenzoic acid)Bos taurus-1 mM289241 2D-image
5,5'-dithiobis(2-nitrobenzoic acid)Bos taurus--289246 2D-image
acetoacetateBos taurus-1 mM, 30% inhibition289242 2D-image
alpha-KetobutyrateBos taurus-1 mM, 50% inhibition289242 2D-image
alpha-ketoglutarateBos taurus-1 mM, 50% inhibition289242 2D-image
BenzoylacetoneBos taurus-9 mM289239 2D-image
Br-Bos taurus-50 mM for 50% inhibition289242 2D-image
citrateBos taurus-9 mM289239 2D-image
Cl-Bos taurus-140 mM for 50% inhibition289242 2D-image
ClO4-Bos taurus-6 mM for 50% inhibition289242 2D-image
DFPBos taurus-1 mM289241 2D-image
DihydroxytartrateBos taurus-1 mM, 100% inhibition289242 2D-image
F-Bos taurus-1.2 mM for 50% inhibition289242 2D-image
Fe3+Bos taurus-inactivation by precipitating the proteins289239 2D-image
HCO3-Bos taurus-170 mM for 50% inhibition289242 2D-image
La3+Bos taurus-inactivation by precipitating the proteins289239 2D-image
N-ethylmaleimideBos taurus-1 mM289241 2D-image
NO3-Bos taurus-12 mM for 50% inhibition289242 2D-image
oxalateBos taurus-9 mM289239 2D-image
oxaloacetateBos taurus-1 mM, 90% inhibition289242 2D-image
p-hydroxymercuribenzoateBos taurus-1 mM289241 2D-image
p-hydroxymercuribenzoateBos taurus--289246 2D-image
pyruvateBos taurus--289242 2D-image
SCN-Bos taurus-4.2 mM for 50% inhibition289242 2D-image
trichloroacetateBos taurus-1.2 mM for 50% inhibition289242 2D-image
Trinitrobenzenesulfonic acidBos taurus-1 mM289241 2D-image
malonateBos taurus-9 mM289239 2D-image
additional informationRattus norvegicus-not inhibited by 2-(2-nitro-4-trifluoromethylbenzoyl)-cyclohexane-1,3-dione670957-
additional informationCaenorhabditis elegansQ94272RNAi directed against the K10C2.4 gene, encoding fumarylacetoacetate hydrolase, produces a lethal phenotype687809-

ACTIVATING COMPOUNDORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE IMAGE
No entries in this field

KM VALUE [mM]KM VALUE [mM] MaximumSUBSTRATEORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE IMAGE
0.27-3,5-hexanedioateBos taurus--289243-
0.22-3-phenylpropionopyruvateBos taurus--289243 2D-image
0.89-5-phenyl-3,5-pentanedioateBos taurus--289243-
0.14-6,6-dimethyl-3,5-heptanedioateBos taurus--289243-
0.47-AcetopyruvateBos taurus--289243 2D-image
0.12-benzoylpyruvateBos taurus--289243 2D-image
0.31-butyropyruvateBos taurus--289243 2D-image
0.021-FumarylacetoacetateHomo sapiens--289247 2D-image
0.81.2FumarylacetoacetateHomo sapiens-liver, dependent on patients289252 2D-image
0.64-pivalopyruvateBos taurus--289243 2D-image
0.47-propionopyruvateBos taurus--289243-
2.36propionopyruvateBos taurus-dependent on pH289246-
1.38-triacetic acidBos taurus--289239 2D-image

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
1.2e-05-3-[(3-carboxy-2-oxopropyl)hydroxyphosphinyl]acrylateHomo sapiens--684964 2D-image
0.085-4-(hydroxymethylphosphinoyl)-3-oxo-butanoic acidHomo sapiens-37°C, recombinant enzyme655997 2D-image
4.1e-05-4-[(2-carboxyethyl)-hydroxyphosphinyl]-3-oxobutyrateHomo sapiens--684964 2D-image

IC50 VALUE [mM]IC50 VALUE [mM] MaximumINHIBITORORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE IMAGE
No entries in this field

SPECIFIC ACTIVITY [µmol/min/mg] SPECIFIC ACTIVITY MAXIMUM ORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE
0.00040.032Homo sapiens-liver, dependent on patients289252
0.018-Oryctolagus cuniculus-kidney289238
0.025-Rattus norvegicus-kidney, 2,4-diketo-6-methyl heptanoate289238
0.026-Felis catus-kidney289238
0.027-Oryctolagus cuniculus-liver289238
0.036-Rattus norvegicus-kidney, 2,4-diketooctanoate289238
0.048-Rattus norvegicus-liver, 2,4-diketo-6-methyl heptanoate289238
0.0523-Rattus norvegicus-kidney, 2,4-diketoheptanoate289238
0.0547-Rattus norvegicus-kidney289238
0.055-Felis catus-liver289238
0.0575-Mus musculus-kidney289238
0.061-Rattus norvegicus-kidney, 2,4-diketo-5,5-dimethyl hexanoate289238
0.0688-Rattus norvegicus-liver289238
0.07-Rattus norvegicus-liver, 2,4-diketooctanoate289238
0.0803-Mus musculus-liver289238
0.0885-Rattus norvegicus-kidney, 2,4-diketononanoate289238
0.0923-Rattus norvegicus-liver, 2,4-diketoheptanoate289238
0.103-Rattus norvegicus-liver, 2,4-diketo-5,5-dimethyl hexanoate289238
0.111-Rattus norvegicus-liver, 2,4-diketoundecanoate289238
0.127-Rattus norvegicus-kidney, 2,4-diketovalerate289238
0.148-Rattus norvegicus-kidney, 2,4-diketo-5-methyl hexanoate289238
0.16-Rattus norvegicus-kidney, 2,4-diketocaprate289238
0.168-Rattus norvegicus-liver, 2,4-diketononanoate289238
0.245-Rattus norvegicus-kidney, 2,4-diketohexanoate289238
0.248-Rattus norvegicus-liver, 2,4-diketo-5-methyl hexanoate289238
0.25-Rattus norvegicus-liver, 2,4-diketovalerate289238
0.337-Rattus norvegicus-liver, 2,4-diketocaprate289238
0.35-Bos taurus--289243
0.395-Rattus norvegicus-liver, 2,4-diketohexanoate289238
0.495-Rattus norvegicus-kidney, 2,4-diketoundecanoate289238
36.3-Bos taurus--289241
69.3-Rattus norvegicus--289245
1558-Homo sapiens--289247

pH OPTIMUMpH MAXIMUMORGANISM UNIPROT ACCESSION NO. COMMENTARYLITERATURE
6.5-Bos taurus-in maleate buffer289239
7-Homo sapiens, Mus musculus-activity assay684964
7.2-Homo sapiens--289247
7.3-Bos taurus--289243
7.57.8Rattus norvegicus-2,4-diketohexanoate289238
88.5Rattus norvegicus-2,4-diketocaprate289238
88.6Rattus norvegicus-2,4-diketononanoate289238
8-Bos taurus-in phosphate and borate buffer289239

pH RANGEpH RANGE MAXIMUMORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
68.5Bos taurus--289239
6.258.5Bos taurus--289246
6.87.6Homo sapiens--289247

TEMPERATURE OPTIMUMTEMPERATURE OPTIMUM MAXIMUMORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
37-Homo sapiens, Mus musculus-assay at655997

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
blood plasmaRattus norvegicus--670957Manually annotated by BRENDA team
brainHomo sapiens, Rattus norvegicus--289253Manually annotated by BRENDA team
erythrocyteHomo sapiens--289248Manually annotated by BRENDA team
fibroblastHomo sapiens--289252Manually annotated by BRENDA team
intestinal epitheliumMus musculus-primary cell699824Manually annotated by BRENDA team
kidneyRattus norvegicus--289238, 289250, 289253Manually annotated by BRENDA team
kidneyFelis catus, Mus musculus, Oryctolagus cuniculus--289238Manually annotated by BRENDA team
kidneyHomo sapiens--289244, 289253Manually annotated by BRENDA team
liverRattus norvegicus--289238, 289240, 289245, 289250, 289253Manually annotated by BRENDA team
liverMus musculus--289238, 289251, 289254, 684964Manually annotated by BRENDA team
liverFelis catus, Oryctolagus cuniculus--289238Manually annotated by BRENDA team
liverBos taurus--289239, 289240, 289241, 289242, 289243, 289246, 657866Manually annotated by BRENDA team
liverHomo sapiens--289244, 289247, 289248, 289249, 289252, 289253, 289255Manually annotated by BRENDA team
proximal tubuleRattus norvegicus--670957Manually annotated by BRENDA team
skin fibroblastHomo sapiens--289256Manually annotated by BRENDA team
spleenMus musculus--289251Manually annotated by BRENDA team

LOCALIZATION ORGANISM UNIPROT ACCESSION NO. COMMENTARY GeneOntology No. LITERATURE SOURCE
intracellularRattus norvegicus--5622289245Manually annotated by BRENDA team
intracellularBos taurus--5622289246Manually annotated by BRENDA team
intracellularHomo sapiens--5622289247Manually annotated by BRENDA team

PDBSCOPCATHORGANISM
1hyo, downloadSCOP (1hyo)CATH (1hyo)Mus musculus
1qcn, downloadSCOP (1qcn)CATH (1qcn)Mus musculus
1qco, downloadSCOP (1qco)CATH (1qco)Mus musculus
1qqj, downloadSCOP (1qqj)CATH (1qqj)Mus musculus
2hzy, downloadSCOP (2hzy)CATH (2hzy)Mus musculus

MOLECULAR WEIGHT MOLECULAR WEIGHT MAXIMUM ORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE
29400-Thermus thermophilus-determined by SDS-PAGE684183
43000-Rattus norvegicus-SDS-PAGE289253
46000-Caenorhabditis elegansQ94272predicted molecular weight, verified by SDS-PAGE and Western blot687809
65000-Arabidopsis thaliana-SDS-PAGE670622
80000-Homo sapiens-SDS-PAGE289249
83000-Homo sapiens-gel filtration289247
86000-Bos taurus-gel filtration289243
87000-Bos taurus-gel filtration289246

SUBUNITS ORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE
dimerBos taurus-2 * 38000-43000, SDS-PAGE, gel filtration, ultracentrifugation289243
dimerBos taurus-2 * 40000-43000, gel filtration289246
dimerHomo sapiens-2 * 41000, SDS-PAGE289247
dimerHomo sapiens-2 * 40000, SDS-PAGE289249
dimerRattus norvegicus-2 * 43000, SDS-PAGE289250
dimerMus musculus-2 * 46000289257
dimerMus musculus-2 * 46000, method not mentioned657866
monomer or dimerArabidopsis thaliana-gel filtration analysis gives an estimated molecular mass of 65000 Da which is an equivalent to 1.4 monomers670622

POSTTRANSLATIONAL MODIFICATION ORGANISM UNIPROT ACCESSION NO. COMMENTARY LITERATURE
No entries in this field

Crystallization/COMMENTARY ORGANISM UNIPROT ACCESSION NO. LITERATURE
-Mus musculus-289257
a high-resolution crystal structure of the FAH-CEHPOBA complex is soved at 1.35 AMus musculus-684964
inhibitor complexed enzyme, 3 mg/ml purified hanging drop vapor diffusion method, room temperature, in a 2:1 ratio with precipitant solution, containing 17-18% PEG 8000, 0.03-0.15 nickel acetate, no to 0.24 M sodium acetate, 0.1 M sodium cacodylate, pH 6.5, soaking in inhibitor 4-(hydroxymethylphosphinoyl)-3-oxo-butanoic acid solution for 16 h prior to data collection via X-ray diffraction, structure determination and analysis at 1.3 A resolutionMus musculus-655997
the native protein is crystallized by the hanging-drop vapor-diffusion method, mixing 1 microl of 15 mg/ml protein solution with 1 microl of crystallization solution at 20°CSulfolobus solfataricus-693645
a recombinant form of the fumarylacetoacetase family member TTHA0809 from Thermus thermophilus HB8 is crystallized, diffraction data are collected at 2.2 AThermus thermophilus-684183

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

TEMPERATURE STABILITYTEMPERATURE STABILITY MAXIMUM ORGANISM UNIPROT ACCESSION NO. COMMENTARYLITERATURE
No entries in this field

GENERAL STABILITYORGANISM UNIPROT ACCESSION NO.LITERATURE
No entries in this field

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
4°C, 1 monthBos taurus-289239
frozen, 3 monthsBos taurus-289239
-70°C, phosphate buffer, pH 7.2, several monthsHomo sapiens-289247
-70°C, several yearsHomo sapiens-289248

Purification/COMMENTARY ORGANISM UNIPROT ACCESSION NO. LITERATURE
nickel-chelate affinity chromatographyArabidopsis thaliana-670622
-Bos taurus-289241, 289242, 289243, 289246
-Homo sapiens-289247, 289248, 289249, 684964
recombinant His-tagged enzymeHomo sapiens-655997
-Mus musculus-289257, 684964
recombinant His-tagged enzyme, fusion protein is cleaved from the fusion tag by bovine thrombinMus musculus-655997
-Rattus norvegicus-289238, 289245, 289250
using a HiPrep 26/10 desalting column, a Super Q Toyopearl 650M column, a Resource Q column, a Bio-Scale CHT-10-I column, and a HiLoad 16/60 Superdex 200 pg columnThermus thermophilus-684183

Cloned/COMMENTARY ORGANISM UNIPROT ACCESSION NO. LITERATURE
expressed in Escherichia coliArabidopsis thaliana-670622
-Homo sapiens-289249
expression in BMT-10 cellsHomo sapiens-289256
expression in CV-1 transfected cellsHomo sapiens-289255
expression of C-terminally His-tagged enzyme in a bacterial expression systemHomo sapiens-655997
for expression in Escherichia coliHomo sapiens-684964
-Mus musculus-289251
expression as glutathione S-transferase fusion proteinMus musculus-655997
for expression in Escherichia coliMus musculus-684964
generation of a Fah-knockout mutant as a model of hereditary tyrosinemia type I. Transplantation of wild-type bone marrow cells from fully allogeneic BALB/c donors into Fah-knockout mutant after lethal total body irradiation results in healthy recipients without pharmacological supportMus musculus-697797
proteome analysis. FAH protein level is down-regulated in the Fe2+ adequate fed TNF-deltaARE mutant and up-regulated in the Fe2+ low fed TNF-deltaARE mutant compared to wild-typeMus musculus-699824
-Rattus norvegicus-289250
into the vector pET11a for expression in Escherichia coli BL21DE3 cellsThermus thermophilus-684183

EXPRESSION ORGANISM UNIPROT ACCESSION NO. LITERATURE
No entries in this field

ENGINEERINGORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
A134DHomo sapiens-mutant with decrased enzyme activity289255
F62CHomo sapiens-mutant with decrased enzyme activity289256
A134DMus musculus--289257
C193RMus musculus-mutant may affect the active-site geometry289257
D233VMus musculus-mutant289257
G158DMus musculus-mutant may affect the active site by perturbing the FAH quarternary structure; mutant may affect the active-site geometry289257
G207DMus musculus-mutant may affect the active-site geometry289257
G369VMus musculus-mutant may affect the active-site geometry289257
N16IMus musculus-mutant may disrupt activity by causing misfolding289257
P249TMus musculus-mutant may affect the active-site geometry289257
P261LMus musculus-mutant may cause general structural effects or misfolding by disrupting the hydrophobic core, the native mainchain conformation and/or important electrostatic interactions289257
P342LMus musculus-mutant may cause general structural effects or misfolding by disrupting the hydrophobic core, the native mainchain conformation and/or important electrostatic interactions289257
S405HMus musculus-mutant may cause general structural effects or misfolding by disrupting the hydrophobic core, the native mainchain conformation and/or important electrostatic interactions289257
V166GMus musculus-mutant may affect the active-site geometry289257
W234GMus musculus-mutant may affect the active-site geometry289257

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

APPLICATIONORGANISM UNIPROT ACCESSION NO.COMMENTARYLITERATURE
biotechnologyArabidopsis thaliana-participation in the synthesis of vitamin E and other tocopherols670622
medicineBos taurus-plays a role in degradation pathway of phenylalanine and tyrosine; plays a role in hereditary tyrosinemia289246
medicineCaenorhabditis elegansQ94272type I tyrosinemia is caused by mutations in the fumarylacetoacetate hydrolase gene, the results establish worms as a model for the study of type I tyrosinemia687809
medicineHomo sapiens-plays a role in hereditary tyrosinemia289244, 289248, 289249
medicineHomo sapiens-plays a role in degradation pathway of phenylalanine and tyrosine; plays a role in hereditary tyrosinemia289247, 289252, 289253, 289255, 289256
medicineHomo sapiens-hereditary tyrosinaemia type 1 results from deficiency of fumarylacetoacetase684964
medicineMus musculus-plays a role in degradation pathway of phenylalanine and tyrosine; plays a role in hereditary tyrosinemia289251, 289257
medicineMus musculus-plays a role in degradation pathway of phenylalanine and tyrosine289254
medicineMus musculus-hereditary tyrosinaemia type 1 results from deficiency of fumarylacetoacetase684964
degradationPseudomonas putida-the enzyme is part of the key catabolic trait for biodegradation of a small number of aromatic compounds669086
medicineRattus norvegicus-plays a role in degradation pathway of phenylalanine and tyrosine289245, 289250, 289253
medicineThermus thermophilus-in humans, deficiency of this activity is associated with the metabolic disease hereditary tyrosinaemia type 1, which is also known as hepatorenal tyrosinaemia684183

DISEASETITLE OF PUBLICATIONLINK TO PUBMED
AdenocarcinomaActivity of flavone acetic acid (NSC-347512) against solid tumors of mice. PubMed
AdenocarcinomaFlavone acetic acid (LM-975; NSC-347512) activation to cytotoxic species in vivo and in vitro. PubMed
AdenocarcinomaFlavone acetic acid antitumour activity against a mouse pancreatic adenocarcinoma is mediated by natural killer cells. PubMed
AdenocarcinomaInfluence of site on the chemosensitivity of transplantable murine colon tumours to flavone acetic acid (LM975, NSC 347512). PubMed
AdenocarcinomaThe ability of coumarin-, flavanon- and flavonol-analogues of flavone acetic acid to stimulate human monocytes. PubMed
Adenoma[Immunohistochemical study in differential diagnosis of benign and malignant lesions of the thyroid gland] PubMed
AlkaptonuriaIn vivo suppressor mutations correct a murine model of hereditary tyrosinemia type I. PubMed
AnemiaFolate status and folate related anemia: a comparative cross-sectional study of long-term care and post-acute care psychogeriatric patients. PubMed
AneurysmClinical relationship between femoral artery aneurysms and arteriomegaly. PubMed
AneurysmIdentification of a chromosome 11q23.2-q24 locus for familial aortic aneurysm disease, a genetically heterogeneous disorder. PubMed
AneurysmRecurrent femoral anastomotic aneurysms. A 30-year experience. PubMed
Aneurysm[Anastomotic pseudoaneurysm, true para-anastomotic aneurysm and recurrent aneurysm following surgery for abdominal aortic aneurysm. Is a unifying theory possible?] PubMed
Arthritis, ExperimentalInverse relationship of hippocampal serotonin to avoidance behavior, serotonergic activation by emotional stress differentiated by estrous cycle and surgical stress. PubMed
Atrial FibrillationElectrophysiologic properties of atrial muscle in paroxysmal atrial fibrillation. PubMed
Bone ResorptionSkeletal lipidomics: regulation of bone metabolism by fatty acid amide family. PubMed
Brain DeathDoes brain death induce a pro-inflammatory response at the organ level in a porcine model? PubMed
Brain InjuriesAttenuation of brain free fatty acid liberation during global ischemia: a model for screening potential therapies for efficacy? PubMed
Brain IschemiaThe influence of MK-801 on the hippocampal free arachidonic acid level and Na+,K+-ATPase activity in global cerebral ischemia-exposed rats. PubMed
Breast NeoplasmsMutation analysis of the Fanconi anaemia A gene in breast tumours with loss of heterozygosity at 16q24.3. PubMed
Breast NeoplasmsNocturnal 5-fluorouracil infusion to patients with breast cancer prior to surgery: appearance of 5-fluorouracil-induced AgNORs aggregation (FAA). PubMed
CarcinomaCombined treatments of heat, radiation, or cytokines with flavone acetic acid on the growth of cultured endothelial cells. PubMed
CarcinomaDifferential efficacy of flavone acetic against liver versus lung metastases in a human tumour xenograft. PubMed
CarcinomaEnhancement of in vitro cytotoxicity of mouse peritoneal exudate cells by flavone acetic acid (NSC 347512). PubMed
CarcinomaEnhancement of the antitumor effect of flavone acetic acid by the bioreductive cytotoxic drug SR 4233 in a murine carcinoma. PubMed
Carcinoma[Immunohistochemical study in differential diagnosis of benign and malignant lesions of the thyroid gland] PubMed
Carcinoma, HepatocellularEffects of carbon tetrachloride and azathioprine on diethylnitrosamine and N-2-fluorenylacetamide-induced hyperplastic liver nodule and hepatocellular carcinoma. PubMed
Carcinoma, HepatocellularEfficient production of Fah-null heterozygote pigs by chimeric adeno-associated virus-mediated gene knockout and somatic cell nuclear transfer. PubMed
Carcinoma, HepatocellularEnzyme deviation patterns in primary rat hepatomas induced by sequential administration of two chemically different carcinogens. PubMed
Carcinoma, HepatocellularRenal function in tyrosinaemia type I after liver transplantation: A long-term follow-up. PubMed
Carcinoma, Lewis LungEnhancement of in vitro cytotoxicity of mouse peritoneal exudate cells by flavone acetic acid (NSC 347512). PubMed
Carcinoma, Papillary[Immunohistochemical study in differential diagnosis of benign and malignant lesions of the thyroid gland] PubMed
Carcinoma, Squamous Cell5,6-dimethylxanthenone-4-acetic acid (DMXAA): a new biological response modifier for cancer therapy. PubMed
Carcinoma, Squamous CellCombined treatments of heat, radiation, or cytokines with flavone acetic acid on the growth of cultured endothelial cells. PubMed
Cell Transformation, NeoplasticPoint mutation instability (PIN) mutator phenotype as model for true back mutations seen in hereditary tyrosinemia type 1 - a hypothesis. PubMed
Colonic NeoplasmsCombination of flavone acetic acid (FAA) with adriamycin, cis-platinum and difluoromethylornithine (DFMO) in vitro against human colon cancer cells. PubMed
Colonic NeoplasmsFlavone acetic acid suppresses human peripheral blood lymphocyte and human colonic lamina propria lymphocyte DNA synthesis. PubMed
Colonic NeoplasmsThe activity of flavone acetic acid (NSC 347512) on human colon cancer cells in vitro. PubMed
Colorectal NeoplasmsResponse to flavone acetic acid (NSC 347512) of primary and metastatic human colorectal carcinoma xenografts. PubMed
Coronary Artery DiseaseA comparison of postmortem coronary atherosclerosis findings in general aviation pilot fatalities. PubMed
CystsDiaphragmatic mesothelial cyst in a child with Fanconi aplastic anemia. PubMed
CystsEchinococcus granulosus: a comparison of free amino acid concentration in hydatid fluid from primary and secondary cysts and host plasma. PubMed
CystsEffect of mebendazole on free amino acid composition of cyst wall and cyst fluid of Echinococcus granulosus harbored in mice. PubMed
DeafnessHearing deficit in a birth cohort of U.S. male commuter air carrier and air taxi pilots. PubMed
DehydrationEffects of fixation and dehydration procedures on marine nematodes. PubMed
Diabetes MellitusFemoral anastomotic aneurysms: a continuing challenge. PubMed
DystoniaDystonia, botulinum neurotoxin, and the aviator. PubMed
Fanconi AnemiaExpression cloning of a cDNA for the major Fanconi anaemia gene, FAA. PubMed,  PubMed
Fanconi AnemiaFunctional activity of the fanconi anemia protein FAA requires FAC binding and nuclear localization. PubMed
Fanconi AnemiaIdentification of Alu-mediated deletions in the Fanconi anemia gene FAA. PubMed
Fanconi AnemiaNormal expression of the Fanconi anemia proteins FAA and FAC and sensitivity to mitomycin C in two patients with Seckel syndrome. PubMed
Fanconi AnemiaSequence variation in the Fanconi anemia gene FAA. PubMed
Fanconi AnemiaThe Fanconi anaemia proteins, FAA and FAC, interact to form a nuclear complex. PubMed
Fanconi AnemiaThe fanconi anemia pathway requires FAA phosphorylation and FAA/FAC nuclear accumulation. PubMed
Fanconi AnemiaThe Fanconi anemia proteins FAA and FAC function in different cellular compartments to protect against cross-linking agent cytotoxicity. PubMed
FibrosarcomaEffect of environmental conditions (pH, oxygenation and temperature) on the cytotoxicity of flavone acetic acid and its dimethylaminoethyl ester. PubMed
FibrosarcomaFlavone acetic acid as a modifier of endothelial cell function. PubMed
fumarylacetoacetase deficiencyA novel mutation causing mild, atypical fumarylacetoacetase deficiency (Tyrosinemia type I): a case report. PubMed
fumarylacetoacetase deficiencyBiochemical studies of a patient with hereditary hepatorenal tyrosinemia: evidence of glutathione deficiency. PubMed
fumarylacetoacetase deficiencyCell fusion is the principal source of bone-marrow-derived hepatocytes. PubMed
fumarylacetoacetase deficiencyCharacterization of a fungal maleylacetoacetate isomerase gene and identification of its human homologue. PubMed
fumarylacetoacetase deficiencyCorrection of fumarylacetoacetate hydrolase deficiency (type I tyrosinemia) in cultured human fibroblasts by retroviral-mediated gene transfer. PubMed
fumarylacetoacetase deficiencyDifferent molecular basis for fumarylacetoacetate hydrolase deficiency in the two clinical forms of hereditary tyrosinemia (type I). PubMed
fumarylacetoacetase deficiencyEffect of nitisinone (NTBC) treatment on the clinical course of hepatorenal tyrosinemia in Québec. PubMed
fumarylacetoacetase deficiencyFumarylacetoacetate, the metabolite accumulating in hereditary tyrosinemia, activates the ERK pathway and induces mitotic abnormalities and genomic instability. PubMed
fumarylacetoacetase deficiencyInduced pluripotent stem cell-derived hepatocytes have the functional and proliferative capabilities needed for liver regeneration in mice. PubMed
fumarylacetoacetase deficiencyMurine fumarylacetoacetate hydrolase (Fah) gene is disrupted by a neonatally lethal albino deletion that defines the hepatocyte-specific developmental regulation 1 (hsdr-1) locus. PubMed
fumarylacetoacetase deficiencySerial transplantation reveals the stem-cell-like regenerative potential of adult mouse hepatocytes. PubMed
fumarylacetoacetase deficiencyTandem mass spectrometric determination of succinylacetone in dried blood spots enables presymptomatic detection in a case of hepatorenal tyrosinaemia. PubMed
fumarylacetoacetase deficiencyThe nephropathy of type I tyrosinemia after liver transplantation. PubMed
fumarylacetoacetase deficiencyThe pathophysiology and treatment of hereditary tyrosinemia type 1. PubMed
fumarylacetoacetase deficiencyThe pre- and post-natal diagnosis of tyrosinemia type I and the detection of the carrier state by assay of fumarylacetoacetase. PubMed
fumarylacetoacetase deficiencyTyrosinaemia--treatment and outcome. PubMed
GliomaDeregulation of ion channel and transporter encoding genes in pediatric gliomas. PubMed
Goiter, Nodular[Immunohistochemical study in differential diagnosis of benign and malignant lesions of the thyroid gland] PubMed
Graves DiseaseEvidence for anti-tubulin autoantibodies in the form of immune complexes in human sera. PubMed
Hashimoto Disease[Immunohistochemical study in differential diagnosis of benign and malignant lesions of the thyroid gland] PubMed
Heart DiseasesRecurrent femoral anastomotic aneurysms. A 30-year experience. PubMed
Heart Failure[False anastomotic aneurysms after aorto-femoral prosthesis. Detection, prevention and treatment] PubMed
HepatitisAutoimmune hepatitis-like disease in C57BL/6 mice infected with mouse hepatitis virus A59. PubMed
HepatitisDetection of mouse hepatitis virus infection by assay of anti-liver autoantibodies. PubMed
HepatitisFine specificity of autoantibodies induced by mouse hepatitis virus A59. PubMed
HepatitisPrevalence of IgG autoantibody against F-actin in patients suspected of having autoimmune or acute viral hepatitis. PubMed
HepatitisSequence similarity and structural homologies are involved in the autoimmune response elicited by mouse hepatitis virus A59. PubMed
HepatitisThe peptide specificities of the autoantibodies elicited by mouse hepatitis virus A59. PubMed
HepatitisUric acid and HMGB1 are involved in the induction of autoantibodies elicited in mice infected with mouse hepatitis virus A59. PubMed
Hepatitis BModeling hepatitis B virus X-induced hepatocellular carcinoma in mice with the sleeping beauty transposon system. PubMed,  PubMed
HypotensionFlavone acetic acid (FAA) with recombinant interleukin-2 (rIL-2) in advanced malignant melanoma: I. Clinical and vascular studies. PubMed
Hypoxia, BrainThe influence of nimodipine and MK-801 on the brain free arachidonic acid level and the learning ability in hypoxia-exposed rats. PubMed
InfectionEffects of starvation and infection with Schistosoma mansoni on the release rate of free amino acids (FAA) by Biomphalaria glabrata. PubMed
Iron OverloadDeferasirox therapy in children with Fanconi aplastic anemia. PubMed
Kidney CalculiThe amino acid factor in stone formers' and normal urines. PubMed
Kidney Failure, ChronicPlasma free amino acids and their metabolites in Taiwanese patients on hemodialysis and continuous ambulatory peritoneal dialysis. PubMed
Kidney NeoplasmsAugmentation of natural killer activity, induction of IFN and development tumor immunity during the successful treatment of established murine renal cancer using flavone acetic acid and IL-2. PubMed
Kidney NeoplasmsCorrelation between in vivo induction of cytokine gene expression by flavone acetic acid and strict dose dependency and therapeutic efficacy against murine renal cancer. PubMed
Kidney NeoplasmsFlavone 8-acetic acid: our current understanding of its mechanism of action in solid tumours. PubMed
Kidney NeoplasmsFlavone-8-acetic acid augments systemic natural killer cell activity and synergizes with IL-2 for treatment of murine renal cancer. PubMed
Kidney NeoplasmsSystemic alkalinization inhibits the ability of flavone acetic acid to augment natural killer activity, induce cytokine gene expression, and synergize with interleukin 2 for the treatment of murine renal cancer. PubMed
LeukemiaActivity of flavone acetic acid (NSC-347512) against solid tumors of mice. PubMed
LeukemiaEffect of flavone acetic acid on Lewis lung carcinoma: evidence for an indirect effect. PubMed
LeukemiaInteraction between flavone acetic acid (LM-975, NSC 349512) and radiation in Glasgow's osteogenic sarcoma in vivo. PubMed
Liver DiseasesAneuploidy as a mechanism for stress-induced liver adaptation. PubMed
Liver DiseasesCell fusion is the principal source of bone-marrow-derived hepatocytes. PubMed
Liver DiseasesHepatocytes corrected by gene therapy are selected in vivo in a murine model of hereditary tyrosinaemia type I. PubMed
Liver DiseasesMyelomonocytic cells are sufficient for therapeutic cell fusion in liver. PubMed
Liver FailureEfficient production of Fah-null heterozygote pigs by chimeric adeno-associated virus-mediated gene knockout and somatic cell nuclear transfer. PubMed
Liver FailureRenal function in tyrosinaemia type I after liver transplantation: A long-term follow-up. PubMed
Liver NeoplasmsAbnormalities in liver iron accumulation during N-2-fluorenylacetamide hepatocarcinogenesis that are dependent or independent of continued carcinogen action. PubMed
Liver NeoplasmsAbsence of a promoting or sequential syncarcinogenic effect in rat liver by the carcinogenic hypolipidemic drug nafenopin given after N-2-fluorenylacetamide. PubMed
Liver NeoplasmsAn enhancing effect of the antihistaminic drug methapyrilene on rat liver carcinogenesis by previously administered N-2-fluorenylacetamide. PubMed
Liver NeoplasmsDose-dependent reduction of N-2-fluorenylacetamide-induced liver cancer and enhancement of bladder cancer in rats by butylated hydroxytoluene. PubMed
Liver NeoplasmsFumarylacetoacetate, the metabolite accumulating in hereditary tyrosinemia, activates the ERK pathway and induces mitotic abnormalities and genomic instability. PubMed
Liver NeoplasmsLack of rapid initiating, promoting or sequential syncarcinogenic effects of di(2-ethylhexyl)phthalate in rat liver carcinogenesis. PubMed
LymphomaEnhancement of in vitro cytotoxicity of mouse peritoneal exudate cells by flavone acetic acid (NSC 347512). PubMed
LymphomaInduction of natural killer cell activity by the antitumour compound flavone acetic acid (NSC 347 512). PubMed
Lymphoma, Non-HodgkinActivity of flavone acetic acid (NSC-347512) against solid tumors of mice. PubMed
MastocytomaEnhancement of in vitro cytotoxicity of mouse peritoneal exudate cells by flavone acetic acid (NSC 347512). PubMed
MelanomaFlavone acetic acid (FAA) with recombinant interleukin-2 (rIL-2) in advanced malignant melanoma: I. Clinical and vascular studies. PubMed
MelanomaFlavone acetic acid induced changes in human endothelial permeability: potentiation by tumour-conditioned medium. PubMed
MelanomaTumour concentrations of flavone acetic acid (FAA) in human melanoma: comparison with mouse data. PubMed
Melanoma, ExperimentalFlavone acetic acid increases the antitumor effect of hyperthermia in mice. PubMed
Melanoma, ExperimentalFlavone acetic acid increases the cytotoxicity of mitomycin C when combined with hyperthermia. PubMed
Melanoma, ExperimentalIn vivo effects of tumor necrosis factor-alpha or flavone acetic acid in combination with doxorubicin on multidrug-resistant B16 melanoma. PubMed
Melanoma, ExperimentalVasoacting agents flavone acetic acid and hydralazine given in combination enhance antitumor effects under condition of hyperthermia. PubMed
Metabolic DiseasesCytoplasmic nonsense-mediated mRNA decay for a nonsense (W262X) transcript of the gene responsible for hereditary tyrosinemia, fumarylacetoacetate hydrolase. PubMed
Metabolic DiseasesFumarylacetoacetate, the metabolite accumulating in hereditary tyrosinemia, activates the ERK pathway and induces mitotic abnormalities and genomic instability. PubMed
Metabolic DiseasesIdentification of a stop mutation in five Finnish patients suffering from hereditary tyrosinemia type I. PubMed
Metabolic DiseasesPharmacological correction of neonatal lethal hepatic dysfunction in a murine model of hereditary tyrosinaemia type I. PubMed
Metabolic DiseasesPoint mutations in the murine fumarylacetoacetate hydrolase gene: Animal models for the human genetic disorder hereditary tyrosinemia type 1. PubMed
Metabolic DiseasesSpectrum of mutations in the fumarylacetoacetate hydrolase gene of tyrosinemia type 1 patients in northwestern Europe and Mediterranean countries. PubMed
Metabolic DiseasesThe mutagenicity of the tyrosine metabolite, fumarylacetoacetate, is enhanced by glutathione depletion. PubMed
Neoplasm MetastasisAugmentation of natural killer activity, induction of IFN and development tumor immunity during the successful treatment of established murine renal cancer using flavone acetic acid and IL-2. PubMed
Neoplasm MetastasisResponse of primary tumour, spontaneous metastases and recurrence of Lewis lung carcinoma (3LL) to flavone acetic acid (FAA, LM975). PubMed
Neoplasm MetastasisResponse to flavone acetic acid (NSC 347512) of primary and metastatic human colorectal carcinoma xenografts. PubMed
Neoplasm, ResidualAugmentation of natural killer activity, induction of IFN and development tumor immunity during the successful treatment of established murine renal cancer using flavone acetic acid and IL-2. PubMed
Neoplasms5,6-dimethylxanthenone-4-acetic acid (DMXAA): a new biological response modifier for cancer therapy. PubMed
NeoplasmsAbsence of a promoting or sequential syncarcinogenic effect in rat liver by the carcinogenic hypolipidemic drug nafenopin given after N-2-fluorenylacetamide. PubMed
NeoplasmsActivity of flavone acetic acid (NSC-347512) against solid tumors of mice. PubMed
NeoplasmsAcute effects of vascular modifying agents in solid tumors assessed by noninvasive laser Doppler flowmetry and near infrared spectroscopy. PubMed
NeoplasmsAnti-tumour activity of flavone acetic acid (NSC 347512) in mice--influence of immune status. PubMed
NeoplasmsAnti-vascular approaches to solid tumour therapy: evaluation of vinblastine and flavone acetic acid. PubMed
NeoplasmsAntitumor activity of chemical modified natural compounds. PubMed
NeoplasmsAntitumor effects of alpha-interferon and gamma-interferon on a murine renal cancer (Renca) in vitro and in vivo. PubMed
NeoplasmsAntivascular approaches to solid tumour therapy: evaluation of tubulin binding agents. PubMed
NeoplasmsAugmentation of natural killer activity, induction of IFN and development tumor immunity during the successful treatment of established murine renal cancer using flavone acetic acid and IL-2. PubMed
NeoplasmsBlood flow failure as a major determinant in the antitumor action of flavone acetic acid. PubMed
NeoplasmsChanges in coagulation and permeability properties of human endothelial cells in vitro induced by TNF-alpha or 5,6 MeXAA. PubMed
NeoplasmsCombined treatments of heat, radiation, or cytokines with flavone acetic acid on the growth of cultured endothelial cells. PubMed
NeoplasmsCorrelation between immune and vascular activities of xanthenone acetic acid antitumor agents. PubMed
NeoplasmsCorrelation between in vivo induction of cytokine gene expression by flavone acetic acid and strict dose dependency and therapeutic efficacy against murine renal cancer. PubMed
NeoplasmsDifferential efficacy of flavone acetic against liver versus lung metastases in a human tumour xenograft. PubMed
NeoplasmsDivergent effects of flavone acetic acid on established versus developing tumour blood flow. PubMed
NeoplasmsEffect of environmental conditions (pH, oxygenation and temperature) on the cytotoxicity of flavone acetic acid and its dimethylaminoethyl ester. PubMed
NeoplasmsEffect of flavone acetic acid (NSC 347,512) on splenic cytotoxic effector cells and their role in tumour necrosis. PubMed
NeoplasmsEffect of flavone acetic acid on endothelial cell proliferation: evidence for antiangiogenic properties. PubMed
NeoplasmsEffect of flavone acetic acid on Lewis lung carcinoma: evidence for an indirect effect. PubMed
NeoplasmsEffects of boron neutron capture therapy using borocaptate sodium in combination with a tumor-selective vasoactive agent in mice. PubMed
NeoplasmsEnhancement of hepatocarcinogenesis by sequential administration of chemicals: summation versus promotion effects. PubMed
NeoplasmsEnhancement of in vitro cytotoxicity of mouse peritoneal exudate cells by flavone acetic acid (NSC 347512). PubMed
NeoplasmsEnhancement of mitomycin-C cytotoxicity by combination with flavone acetic acid in a murine tumour. PubMed
NeoplasmsEnhancement of radioimmunotherapy by drugs modifying tumour blood flow in a colonic xenograft model. PubMed
NeoplasmsEnhancement of the antitumor effect of flavone acetic acid by the bioreductive cytotoxic drug SR 4233 in a murine carcinoma. PubMed
NeoplasmsEvidence for the production of nitric oxide by activated macrophages treated with the antitumor agents flavone-8-acetic acid and xanthenone-4-acetic acid. PubMed
NeoplasmsFlavone 8-acetic acid: our current understanding of its mechanism of action in solid tumours. PubMed
NeoplasmsFlavone acetic acid (FAA) with recombinant interleukin-2 (rIL-2) in advanced malignant melanoma. IV: Pharmacokinetics and toxicity of flavone acetic acid and its metabolites. PubMed
NeoplasmsFlavone acetic acid (FAA) with recombinant interleukin-2 (rIL-2) in advanced malignant melanoma: I. Clinical and vascular studies. PubMed
NeoplasmsFlavone acetic acid (LM-975; NSC-347512) activation to cytotoxic species in vivo and in vitro. PubMed
NeoplasmsFlavone acetic acid (NSC 347512)-induced modulation of murine tumor physiology monitored by in vivo nuclear magnetic resonance spectroscopy. PubMed
NeoplasmsFlavone acetic acid and plasma protein binding. PubMed
NeoplasmsFlavone acetic acid antitumour activity against a mouse pancreatic adenocarcinoma is mediated by natural killer cells. PubMed
NeoplasmsFlavone acetic acid as a modifier of endothelial cell function. PubMed
NeoplasmsFlavone acetic acid directly induces expression of cytokine genes in mouse splenic leukocytes but not in human peripheral blood leukocytes. PubMed
NeoplasmsFlavone acetic acid distribution in human malignant tumors. PubMed
NeoplasmsFlavone acetic acid increases the antitumor effect of hyperthermia in mice. PubMed
NeoplasmsFlavone acetic acid increases the cytotoxicity of mitomycin C when combined with hyperthermia. PubMed
NeoplasmsFlavone acetic acid induced changes in human endothelial permeability: potentiation by tumour-conditioned medium. PubMed
NeoplasmsFlavone acetic acid induces a coagulopathy in mice. PubMed
NeoplasmsFlavone acetic acid pharmacokinetics in nude mice. PubMed
NeoplasmsFlavone acetic acid potentiates the induction of endothelial procoagulant activity by tumour necrosis factor. PubMed
NeoplasmsFlavone acetic acid stimulates nitric oxide and peroxynitrite production in subcutaneous mouse tumors. PubMed
NeoplasmsFlavone-8-acetic acid augments systemic natural killer cell activity and synergizes with IL-2 for treatment of murine renal cancer. PubMed
NeoplasmsHepatocellular carcinoma despite long-term survival in chronic tyrosinaemia I. PubMed
NeoplasmsImmunological effects of flavone acetic acid. PubMed
NeoplasmsInadequate vasculature in solid tumours: consequences for cancer research strategies. PubMed
NeoplasmsInduction of hypoxia in the KHT sarcoma by tumour necrosis factor and flavone acetic acid. PubMed
NeoplasmsInduction of multiple cytokine gene expression and IRF-1 mRNA by flavone acetic acid in a murine macrophage cell line. PubMed
NeoplasmsInduction of natural killer activity by xanthenone analogues of flavone acetic acid: relation with antitumour activity. PubMed
NeoplasmsInduction of natural killer cell activity by the antitumour compound flavone acetic acid (NSC 347 512). PubMed
NeoplasmsInduction of tumour hypoxia by FAA and TNF: interaction with bioreductive drugs. PubMed
NeoplasmsInfluence of site on the chemosensitivity of transplantable murine colon tumours to flavone acetic acid (LM975, NSC 347512). PubMed
NeoplasmsInhibition of antitumor effects of flavone acetic acid by cortisone. PubMed
NeoplasmsInhibitory effect of ellagic acid on N-2-fluorenylacetamide-induced liver carcinogenesis in male ACI/N rats. PubMed
NeoplasmsInteraction between flavone acetic acid (LM-975, NSC 349512) and radiation in Glasgow's osteogenic sarcoma in vivo. PubMed
NeoplasmsLong-term effects of flavone acetic acid on the growth of a rat tumour. PubMed
NeoplasmsMechanisms of the inhibitory action of p-hydroxyacetanilide on carcinogenesis by N-2-fluorenylacetamide or N-hydroxy-N-2-fluorenylacetamide. PubMed
NeoplasmsNitric oxide: its production in host-cell-infiltrated EMT6 spheroids and its role in tumour cell killing by flavone-8-acetic acid and 5,6-dimethylxanthenone-4-acetic acid. PubMed
NeoplasmsNocturnal 5-fluorouracil infusion to patients with breast cancer prior to surgery: appearance of 5-fluorouracil-induced AgNORs aggregation (FAA). PubMed
NeoplasmsNucleolar organizer regions in hepatocarcinogenesis induced by N-2-fluorenylacetamide in rats: comparison with bromodeoxyuridine immunohistochemistry. PubMed
NeoplasmsOxidative stress contributes to the anti-proliferative effects of flavone acetic acid on endothelial cells. PubMed
NeoplasmsPharmacodynamics and causes of dose-dependent pharmacokinetics of flavone-8-acetic acid (LM-975; NSC-347512) in mice. PubMed
NeoplasmsPotentiation of the cytotoxicity of carboquone by flavone acetic acid combined with hyperthermia. PubMed
NeoplasmsPreclinical in vitro and in vivo activity of 5,6-dimethylxanthenone-4-acetic acid. PubMed
NeoplasmsProduction of tumour necrosis factor-alpha by cultured human peripheral blood leucocytes in response to the anti-tumour agent 5,6-dimethylxanthenone-4-acetic acid (NSC 640488). PubMed
NeoplasmsRecombinant interleukin-2 (rIL-2) with flavone acetic acid (FAA) in advanced malignant melanoma: a phase II study. PubMed
NeoplasmsReduction of cytotoxic effector cell activity in colon 38 tumours following treatment with flavone acetic acid. PubMed
NeoplasmsReduction of tumor blood flow by flavone acetic acid: a possible component of therapy. PubMed
NeoplasmsResponse of chemically induced primary colon tumours of the mouse to flavone acetic acid (NSC 347 512). PubMed
NeoplasmsResponse of primary tumour, spontaneous metastases and recurrence of Lewis lung carcinoma (3LL) to flavone acetic acid (FAA, LM975). PubMed
NeoplasmsResponse to flavone acetic acid (NSC 347512) of primary and metastatic human colorectal carcinoma xenografts. PubMed
NeoplasmsResponses of experimental rat tumours and a mouse colon tumour to flavone acetic acid. PubMed
NeoplasmsReview article: angiogenesis, neovascular proliferation and vascular pathophysiology as targets for cancer therapy. PubMed
NeoplasmsRole of T cells and tumour necrosis factor in antitumour activity and toxicity of flavone acetic acid. PubMed
NeoplasmsRole of tumor necrosis factor in flavone acetic acid-induced tumor vasculature shutdown. PubMed
NeoplasmsSelective induction of endothelial cell tissue factor in the presence of a tumour-derived mediator: a potential mechanism of flavone acetic acid action in tumour vasculature. PubMed
NeoplasmsSerotonin involvement in the antitumour and host effects of flavone-8-acetic acid and 5,6-dimethylxanthenone-4-acetic acid. PubMed
NeoplasmsSmall-molecule cytokine inducers causing tumor necrosis. PubMed
NeoplasmsStudies with a panel of tumours having a variable sensitivity to FAA, to investigate its mechanism of action. PubMed
NeoplasmsSynthesis and antitumour activity of new derivatives of flavone-8-acetic acid (FAA). Part 1: 6-Methyl derivatives. PubMed
NeoplasmsSystemic alkalinization inhibits the ability of flavone acetic acid to augment natural killer activity, induce cytokine gene expression, and synergize with interleukin 2 for the treatment of murine renal cancer. PubMed
NeoplasmsSystemic effects of FAA are enhanced by implanted tumours. PubMed
NeoplasmsTesticular mesotheliomas in rats exposed to N-2-fluorenylacetamide (FAA). PubMed
NeoplasmsThe effect of 5,6-dimethylxanthenone-4-acetic acid on tumour necrosis factor production by human immune cells. PubMed
NeoplasmsThe effect of blood flow modification on intra- and extracellular pH measured by 31P magnetic resonance spectroscopy in murine tumours. PubMed
NeoplasmsThe in vivo interaction between flavone acetic acid and hyperthermia. PubMed
NeoplasmsThe relationship between tissue levels of flavone acetic acid (NSC 347512) and site dependent anti-tumour activity in murine colon tumours. PubMed
NeoplasmsThe role of immune effector cells in flavone acetic acid-induced injury to tumor cells in EMT6 spheroids. PubMed
NeoplasmsThe Sir Mark Oliphant Conferences: international frontiers of science and technology. PubMed
NeoplasmsThe use of vascularised spheroids to investigate the action of flavone acetic acid on tumour blood vessels. PubMed
NeoplasmsTumor-dependent increased plasma nitrate concentrations as an indication of the antitumor effect of flavone-8-acetic acid and analogues in mice. PubMed
NeoplasmsTumour concentrations of flavone acetic acid (FAA) in human melanoma: comparison with mouse data. PubMed
NeoplasmsUnique chemosensitivity of MAC 16 tumours to flavone acetic acid (LM975, NSC 347512). PubMed
NeoplasmsVascular attack by 5,6-dimethylxanthenone-4-acetic acid combined with B7.1 (CD80)-mediated immunotherapy overcomes immune resistance and leads to the eradication of large tumors and multiple tumor foci. PubMed
NeoplasmsVascular collapse after flavone acetic acid: a possible mechanism of its anti-tumour action. PubMed
NeoplasmsVasoacting agents flavone acetic acid and hydralazine given in combination enhance antitumor effects under condition of hyperthermia. PubMed
ObesityCo-morbid conditions in overweight and obese airmen: trends and aeromedical implications. PubMed
OsteoporosisSkeletal lipidomics: regulation of bone metabolism by fatty acid amide family. PubMed
OsteosarcomaFlavone acetic acid (NSC 347512)-induced DNA damage in Glasgow osteogenic sarcoma in vivo. PubMed
OsteosarcomaFlavone acetic acid (NSC 347512)-induced modulation of murine tumor physiology monitored by in vivo nuclear magnetic resonance spectroscopy. PubMed
OsteosarcomaInteraction between flavone acetic acid (LM-975, NSC 349512) and radiation in Glasgow's osteogenic sarcoma in vivo. PubMed
PainFunctional disassociation of the central and peripheral fatty acid amide signaling systems. PubMed
PainThe enzymatic inactivation of the fatty acid amide class of signaling lipids. PubMed
Porphyria, Acute IntermittentRenal function in tyrosinaemia type I after liver transplantation: A long-term follow-up. PubMed
PorphyriasRenal function in tyrosinaemia type I after liver transplantation: A long-term follow-up. PubMed
Porphyrias[delta-Aminolevulinate dehydratase deficiency] PubMed
Porphyrias, Hepatic[delta-Aminolevulinate dehydratase deficiency] PubMed
Pregnancy, High-RiskFumarylacetoacetase activity in cultured and non-cultured chorionic villus cells, and assay in two high-risk pregnancies. PubMed
Pulmonary Heart DiseaseElectrophysiological study of infant and adult rats under acute intoxication with fluoroacetamide. PubMed
Renal Insufficiency[False anastomotic aneurysms after aorto-femoral prosthesis. Detection, prevention and treatment] PubMed
RhabdomyosarcomaResponses of experimental rat tumours and a mouse colon tumour to flavone acetic acid. PubMed
SarcomaActivity of flavone acetic acid (NSC-347512) against solid tumors of mice. PubMed
SarcomaInduction of hypoxia in the KHT sarcoma by tumour necrosis factor and flavone acetic acid. PubMed
SarcomaLong-term effects of flavone acetic acid on the growth of a rat tumour. PubMed
SeizuresMerging the structural motifs of functionalized amino acids and alpha-aminoamides: compounds with significant anticonvulsant activities. PubMed
Sepsis[False anastomotic aneurysms after aorto-femoral prosthesis. Detection, prevention and treatment] PubMed
StarvationEffect of starvation on free histidine and amino acids in white muscle of milkfish Chanos chanos. PubMed
Starvation[A study on the constitutional change of taurine and other free amino acids during the fetal development (author's transl)] PubMed
StrokeClinical evaluation of a fully automated model-based algorithm to calculate left ventricular volumes and ejection fraction using multidetector computed tomography. PubMed
Testicular NeoplasmsTesticular tumor in an aviator. PubMed
ThrombosisFlavone-8-acetic acid (Flavonoid) profoundly reduces platelet-dependent thrombosis and vasoconstriction after deep arterial injury In vivo. PubMed
TyrosinemiasA GC/MS validated method for the nanomolar range determination of succinylacetone in amniotic fluid and plasma: An analytical tool for tyrosinemia type I. PubMed
TyrosinemiasA missense mutation (Q279R) in the fumarylacetoacetate hydrolase gene, responsible for hereditary tyrosinemia, acts as a splicing mutation. PubMed
TyrosinemiasA mouse model of renal tubular injury of tyrosinemia type 1: development of de Toni Fanconi syndrome and apoptosis of renal tubular cells in Fah/Hpd double mutant mice. PubMed
TyrosinemiasA novel mutation causing mild, atypical fumarylacetoacetase deficiency (Tyrosinemia type I): a case report. PubMed
TyrosinemiasA single mutation of the fumarylacetoacetate hydrolase gene in French Canadians with hereditary tyrosinemia type I. PubMed
TyrosinemiasAAV Vectors Containing rDNA Homology Display Increased Chromosomal Integration and Transgene Persistence. PubMed
TyrosinemiasAdenovirus-mediated gene therapy in a mouse model of hereditary tyrosinemia type I. PubMed
TyrosinemiasAnimal models reveal pathophysiologies of tyrosinemias. PubMed
TyrosinemiasBiochemical studies on the enzymatic deficiencies in hereditary tyrosinemia. PubMed
TyrosinemiasCharacterization of a fungal maleylacetoacetate isomerase gene and identification of its human homologue. PubMed
TyrosinemiasCharacterization of the human fumarylacetoacetate hydrolase gene and identification of a missense mutation abolishing enzymatic activity. PubMed
TyrosinemiasCloning and expression analysis of a cDNA encoding fumarylacetoacetate hydrolase: post-transcriptional modulation in rat liver and kidney. PubMed
TyrosinemiasCloning and expression of the cDNA encoding human fumarylacetoacetate hydrolase, the enzyme deficient in hereditary tyrosinemia: assignment of the gene to chromosome 15. PubMed
TyrosinemiasCompound mutations (R237X and L375P) in the fumarylacetoacetate hydrolase gene causing tyrosinemia type I in a Chinese patient. PubMed
TyrosinemiasCorrection of fumarylacetoacetate hydrolase deficiency (type I tyrosinemia) in cultured human fibroblasts by retroviral-mediated gene transfer. PubMed
TyrosinemiasCurrent strategies for the treatment of hereditary tyrosinemia type I. PubMed
TyrosinemiasCytoplasmic nonsense-mediated mRNA decay for a nonsense (W262X) transcript of the gene responsible for hereditary tyrosinemia, fumarylacetoacetate hydrolase. PubMed
TyrosinemiasDeficiency of fumarylacetoacetase in a patient with hereditary tyrosinemia. PubMed
TyrosinemiasDeficiency of fumarylacetoacetase without hereditary tyrosinemia. PubMed
TyrosinemiasDeficient DNA-ligase activity in the metabolic disease tyrosinemia type I. PubMed
TyrosinemiasDiagnosis and management of tyrosinemia type I. PubMed
TyrosinemiasDifferent clinical forms of hereditary tyrosinemia (type I) in patients with identical genotypes. PubMed
TyrosinemiasDifferent molecular basis for fumarylacetoacetate hydrolase deficiency in the two clinical forms of hereditary tyrosinemia (type I). PubMed
TyrosinemiasDNA damage and repair in mammalian cells exposed to p-hydroxyphenylpyruvic acid. PubMed
TyrosinemiasEffect of nitisinone (NTBC) treatment on the clinical course of hepatorenal tyrosinemia in Québec. PubMed
TyrosinemiasEfficient production of Fah-null heterozygote pigs by chimeric adeno-associated virus-mediated gene knockout and somatic cell nuclear transfer. PubMed
TyrosinemiasEvaluation of dichloroacetate treatment in a murine model of hereditary tyrosinemia type 1. PubMed
TyrosinemiasExpression and post-translational modification of human 4-hydroxy-phenylpyruvate dioxygenase. PubMed
TyrosinemiasExtensive changes in liver gene expression induced by hereditary tyrosinemia type I are not normalized by treatment with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC). PubMed
TyrosinemiasFrequent mutation reversion inversely correlates with clinical severity in a genetic liver disease, hereditary tyrosinemia. PubMed
TyrosinemiasFumarylacetoacetase measurement as a mass-screening procedure for hereditary tyrosinemia type I. PubMed
TyrosinemiasFumarylacetoacetate, the metabolite accumulating in hereditary tyrosinemia, activates the ERK pathway and induces mitotic abnormalities and genomic instability. PubMed
TyrosinemiasGene expression profiles of homogentisate-treated Fah-/- Hpd-/-mice using DNA microarrays. PubMed
TyrosinemiasHarnessing a high cargo-capacity transposon for genetic applications in vertebrates. PubMed
TyrosinemiasHepatocyte injury in tyrosinemia type 1 is induced by fumarylacetoacetate and is inhibited by caspase inhibitors. PubMed
TyrosinemiasHereditary tyrosinemia type 1 metabolites impair DNA excision repair pathways. PubMed
TyrosinemiasHereditary tyrosinemia type 1: novel missense, nonsense and splice consensus mutations in the human fumarylacetoacetate hydrolase gene; variability of the genotype-phenotype relationship. PubMed
TyrosinemiasHereditary tyrosinemia type I. Self-induced correction of the fumarylacetoacetase defect. PubMed
TyrosinemiasHereditary tyrosinemia type I: lack of correlation between clinical findings and amount of immunoreactive fumarylacetoacetase protein. PubMed
TyrosinemiasHereditary tyrosinemia type I: strong association with haplotype 6 in French Canadians permits simple carrier detection and prenatal diagnosis. PubMed
TyrosinemiasHereditary tyrosinemias (type I): a new vista on tyrosine toxicity and cancer. PubMed
TyrosinemiasHigh volume naked DNA tail-vein injection restores liver function in Fah-knock out mice. PubMed
TyrosinemiasIdentification of a frequent pseudodeficiency mutation in the fumarylacetoacetase gene, with implications for diagnosis of tyrosinemia type I. PubMed
TyrosinemiasIdentification of a stop mutation in five Finnish patients suffering from hereditary tyrosinemia type I. PubMed
TyrosinemiasIdentification of mutations causing hereditary tyrosinemia type I in patients of Middle Eastern origin. PubMed
TyrosinemiasImaging features of type 1 hereditary tyrosinemia: a review of 30 patients. PubMed
TyrosinemiasImpaired DNA repair and genomic stability in hereditary tyrosinemia type 1. PubMed
TyrosinemiasIn vivo correction of murine hereditary tyrosinemia type I by phiC31 integrase-mediated gene delivery. PubMed
TyrosinemiasIn vivo selection of hepatocytes transduced with adeno-associated viral vectors. PubMed
TyrosinemiasIn vivo suppressor mutations correct a murine model of hereditary tyrosinemia type I. PubMed
TyrosinemiasInvolvement of endoplasmic reticulum stress in hereditary tyrosinemia type I. PubMed
TyrosinemiasLC-MS/MS method for simultaneous determination on a dried blood spot of multiple analytes relevant for treatment monitoring in patients with tyrosinemia type I. PubMed
TyrosinemiasLiver repopulation and correction of metabolic liver disease by transplanted adult mouse pancreatic cells. PubMed
TyrosinemiasLocalization of cells in the rat brain expressing fumarylacetoacetate hydrolase, the deficient enzyme in hereditary tyrosinemia type 1. PubMed
TyrosinemiasLong-term outcome of living donor liver transplantation in a Thai boy with hereditary tyrosinemia type I: a case report. PubMed
TyrosinemiasMurine fumarylacetoacetate hydrolase (Fah) gene is disrupted by a neonatally lethal albino deletion that defines the hepatocyte-specific developmental regulation 1 (hsdr-1) locus. PubMed
TyrosinemiasMutations in the fumarylacetoacetate hydrolase gene causing hereditary tyrosinemia type I: overview. PubMed
TyrosinemiasMutations of the fumarylacetoacetate hydrolase gene in four patients with tyrosinemia, type I. PubMed
TyrosinemiasNo evidence of maternal cell colonization in reverted liver nodules of tyrosinemia type I patients. PubMed
TyrosinemiasNovel splice, missense, and nonsense mutations in the fumarylacetoacetase gene causing tyrosinemia type 1. PubMed
TyrosinemiasNucleotide sequence of a cDNA encoding murine fumarylacetoacetate hydrolase. PubMed
TyrosinemiasOn the enzymic defects in hereditary tyrosinemia. PubMed
TyrosinemiasPoint mutation instability (PIN) mutator phenotype as model for true back mutations seen in hereditary tyrosinemia type 1 - a hypothesis. PubMed
TyrosinemiasPoint mutations in the murine fumarylacetoacetate hydrolase gene: Animal models for the human genetic disorder hereditary tyrosinemia type 1. PubMed
TyrosinemiasPrenatal diagnosis of hereditary tyrosinemia by determination of fumarylacetoacetase in cultured amniotic fluid cells. PubMed
TyrosinemiasPurification of mRNA coding for the enzyme deficient in hereditary tyrosinemia, fumarylacetoacetate hydrolase. PubMed
TyrosinemiasRenal proximal tubular cells acquire resistance to cell death stimuli in mice with hereditary tyrosinemia type 1. PubMed
TyrosinemiasRescue from neonatal death in the murine model of hereditary tyrosinemia by glutathione monoethylester and vitamin C treatment. PubMed
TyrosinemiasSelf-induced correction of the genetic defect in tyrosinemia type I. PubMed
TyrosinemiasSimple and fast quantification of nitisone (NTBC) using liquid chromatography-tandem mass spectrometry method in plasma of tyrosinemia type 1 patients. PubMed
TyrosinemiasSix novel mutations in the fumarylacetoacetate hydrolase gene of patients with hereditary tyrosinemia type I. PubMed
TyrosinemiasSpectrum of mutations in the fumarylacetoacetate hydrolase gene of tyrosinemia type 1 patients in northwestern Europe and Mediterranean countries. PubMed
TyrosinemiasSplicing mutations, mainly IVS6-1(G>T), account for 70% of fumarylacetoacetate hydrolase (FAH) gene alterations, including 7 novel mutations, in a survey of 29 tyrosinemia type I patients. PubMed
TyrosinemiasStructural and functional analysis of missense mutations in fumarylacetoacetate hydrolase, the gene deficient in hereditary tyrosinemia type 1. PubMed
TyrosinemiasStructural organization and analysis of the human fumarylacetoacetate hydrolase gene in tyrosinemia type I. PubMed
TyrosinemiasSuccinylacetone inhibits delta-aminolevulinate dehydratase and potentiates the drug and steroid induction of delta-aminolevulinate synthase in liver. PubMed
TyrosinemiasTandem mass spectrometric determination of succinylacetone in dried blood spots enables presymptomatic detection in a case of hepatorenal tyrosinaemia. PubMed
TyrosinemiasThe Caenorhabditis elegans K10C2.4 Gene Encodes a Member of the Fumarylacetoacetate Hydrolase Family: A CAENORHABDITIS ELEGANS MODEL OF TYPE I TYROSINEMIA. PubMed
TyrosinemiasThe effects of early treatment of hereditary tyrosinemia type I in infancy by orthotopic liver transplantation. PubMed
TyrosinemiasThe human fumarylacetoacetase gene: characterisation of restriction fragment length polymorphisms and identification of haplotypes in tyrosinemia type 1 and pseudodeficiency. PubMed
TyrosinemiasThe kidney in children with tyrosinemia: sonographic, CT and biochemical findings. PubMed
TyrosinemiasThe mutagenicity of the tyrosine metabolite, fumarylacetoacetate, is enhanced by glutathione depletion. PubMed
TyrosinemiasThe nephropathy of type I tyrosinemia after liver transplantation. PubMed
TyrosinemiasThe pathophysiology and treatment of hereditary tyrosinemia type 1. PubMed
TyrosinemiasThe pre- and post-natal diagnosis of tyrosinemia type I and the detection of the carrier state by assay of fumarylacetoacetase. PubMed
TyrosinemiasTwo missense mutations causing tyrosinemia type 1 with presence and absence of immunoreactive fumarylacetoacetase. PubMed
TyrosinemiasType 1 hereditary tyrosinemia. Evidence for molecular heterogeneity and identification of a causal mutation in a French Canadian patient. PubMed
TyrosinemiasType I tyrosinemia: lack of immunologically detectable fumarylacetoacetase enzyme protein in tissues and cell extracts. PubMed
TyrosinemiasTyrosinaemia--treatment and outcome. PubMed
TyrosinemiasTyrosinemia type 1 should be suspected in infants with severe coagulopathy even in the absence of other signs of liver failure. PubMed
TyrosinemiasTyrosinemia type 1--complex splicing defects and a missense mutation in the fumarylacetoacetase gene. PubMed
TyrosinemiasTyrosinemia type I: a clinico-laboratory case report. PubMed
TyrosinemiasTyrosinemia: the Quebec experience. PubMed
TyrosinemiasUrinary excretion of deuterated metabolites in patients with tyrosinemia type I after oral loading with deuterated L-tyrosine. PubMed
TyrosinemiasUrinary excretion of succinylacetone and delta-aminolevulinic acid in patients with hereditary tyrosinemia. PubMed
TyrosinemiasVariable gene expression within human tyrosinemia type 1 liver may reflect region-specific dysplasia. PubMed
Tyrosinemias[Clinical, biochemical and molecular characteristics in 11 Czech children with tyrosinemia type I]. PubMed
Tyrosinemias[delta-Aminolevulinate dehydratase deficiency] PubMed
Tyrosinemias[Evolution of a case of tyrosinemia type I treated with NTBC] PubMed
Tyrosinemias[Juvenile form of tyrosinemia type I] PubMed
Urinary Bladder NeoplasmsDose-dependent reduction of N-2-fluorenylacetamide-induced liver cancer and enhancement of bladder cancer in rats by butylated hydroxytoluene. PubMed
VertigoBenign paroxysmal positional vertigo in an airline pilot. PubMed

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289238Meister, A.; Greenstein, J.P.Enzymatic hydrolysis of 2,4-diketo acidsJ. Biol. Chem.175573-5881948Felis catus, Mus musculus, Oryctolagus cuniculus, Rattus norvegicus PubMed
289239Connors, W.M.; Stotz, E.The purification and properties of a triacetic acid-hydrolyzing enzymeJ. Biol. Chem.178881-8901949Bos taurus PubMed
289240Edwards, S.W.; Knox, W.E.Homogentisate metabolism: The isomerization of meleylacetoacetate by an enzyme which requires glutathioneJ. Biol. Chem.22079-911956Bos taurus, Rattus norvegicus PubMed
289241Hsiang,.H.; Sim, S.S.; Mahuran, D.J.; Schmidt, Jr., D.E.Purification and properties of a diketo acid hydrolase from beef liverBiochemistry112098-21021972Bos taurus PubMed
289242Braun, C.V.; Schmidt, Jr., D.E.Inhibition of fumarylacetoacetate fumarylhydrolase by monovalent anionsBiochemistry124878-48811973Bos taurus PubMed
289243Mahuran, D.J.; Angus, R.H.; Braun, C.V.; Sim, S.S.; Schmidt, Jr., D.E.Characterization and substrate specificity of fumarylacetoacetate fumarylhydrolaseCan. J. Biochem.551-81977Bos taurus PubMed
289244Lindblad, B.; Lindstedt, S.; Steen, G.On the enzymic defects in hereditary tyrosinemiaProc. Natl. Acad. Sci. USA744641-46451977Homo sapiens PubMed
289245Doshi, K.S.; Schmidt, Jr., D.E.Localization of fumarylacetoacetate fumarylhydrolase in rat liverCan. J. Biochem.56866-8681977Rattus norvegicus-
289246Nagainis, M.P.; Pu, W.; Cheng, B.; Taylor, K.E.; Schmidt, Jr., D.E.Effects of pH and sulfhydryl specific reagents on 4-fumarylacetoacetate fumarylhydrolaseBiochim. Biophys. Acta657203-2111981Bos taurus PubMed
289247Van Faassen, H.; van den Berg, I.E.T.; Berger, R.Purification of human liver fumarylacetoacetase using immunoaffinity chromatographyJ. Biochem. Biophys. Methods20317-3241990Homo sapiens-
289248Laberge, C.; Grenier, A.; Valet, J.P.; Morissette, J.Fumarylacetoacetase measurement as a mass-screening procedure for hereditary tyrosinemia type IAm. J. Hum. Genet.47325-3281990Homo sapiens PubMed
289249Phaneuf, D.; Labelle, Y.; Berube, D.; Arden, K.; Cavenee, W.; Gagne, R; Tanguay, R.M.Cloning and expression of the cDNA encoding human fumarylacetoacetate hydrolase, the enzyme deficient in hereditary tyrosinemia: Assignment of the gene to chromosome 15Am. J. Hum. Genet.48525-5351991Homo sapiens PubMed
289250Labelle, Y.; Phaneuf, D.; Tanguay, R.M.Cloning and expression analysis of a cDNA encoding fumarylacetoacetate hydrolase: post-transcriptional modulation in rat liver and kidneyGene104197-2021991Rattus norvegicus PubMed
289251Klebig, M.L.; Russell, L.B.; Rinchik, E.M.Murine fumarylacetoacetate hydrolase (Fah) gene is disrupted by a neonatally lethal albino deletion that defines the hepatocyte-specific developmental regulation 1 (hsdr-1) locusProc. Natl. Acad. Sci. USA891363-13671992Mus musculus PubMed
289252Kvittingen, E.A.; Rootwelt, H.; Brandtzaeg, P.; Bergan, A.; Berger, R.Hereditary tyrosinemia type IJ. Clin. Invest.911816-18211993Homo sapiens PubMed
289253Labelle, Y.; Puymirat, J.; Tanguay, R.M.Localization of cells in the rat brain expressing fumarylacetoacetate hydrolase, the deficient enzyme in hereditary tyrosinemia type 1Biochim. Biophys. Acta1180250-2561993Homo sapiens, Rattus norvegicus PubMed
289254Grompe, M.; Al-Dhalimy, M.; Finegold, M.; Ou, C.N.; Burlingame, T.; Kennaway, N.G.; Soriano, P.Loss of fumarylacetoacetate hydrolysis is responsible for the neonatal hepatic dysfunction phenotype of lethal albino miceGenes Dev.72298-23071993Mus musculus PubMed
289255Labelle, Y.; Phaneuf, D.; Leclerc, B.; Tanguay, R.M.Characterization of the human fumarylacetoacetate hydrolase gene and identification of a missense mutation abolishing enzymatic activityHum. Mol. Genet.2941-9461993Homo sapiens PubMed
289256Awata, H.; Endo, F.; Tanoue, A.; Kitano, A.; Nakano, Y.; Matsuda, I.Structural organization and analysis of the human fumarylacetoacetate hydrolase gene in tyrosinemia type IBiochim. Biophys. Acta1226168-1721994Homo sapiens PubMed
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LINKS TO OTHER DATABASES (specific for EC-Number 3.7.1.2)
ExplorEnz
ExPASy
KEGG
MetaCyc
NCBI: PubMed, Protein, Nucleotide, Structure, Genome, OMIM
IUBMB Enzyme Nomenclature
PROSITE Database of protein families and domains
SYSTERS
Protein Mutant Database
InterPro (database of protein families, domains and functional sites)