Fumarylacetoacetate hydrolase

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fumarylacetoacetate hydrolase (fumarylacetoacetase)
Identifiers
Symbol	FAH
Entrez	2184
HUGO	3579
OMIM	276700
RefSeq	NM_000137
UniProt	P16930
Other data
EC number	3.7.1.2
Locus	Chr. 15 q23-q25

Fumarylacetoacetate hydrolase is an enzyme which catalyzes the hydrolysis of 4-fumarylacetoacetate into fumarate and acetoacetate.

Pathology

Mutations in the FAH gene cause type I tyrosinemia.

External links

• MeSH fumarylacetoacetase

Template:Enzyme-stub

v • d • e Carbon-carbon hydrolases (EC 3.7)
Fumarylacetoacetate hydrolase - Kynureninase

v • d • e Amino acid metabolism enzymes (essential in CAPS)
K	LYSINE→acetyl-CoA Saccharopine dehydrogenase - Glutaryl-CoA dehydrogenase LEUCINE→acetyl-CoA Branched-chain alpha-keto acid dehydrogenase complex - Isovaleryl coenzyme A dehydrogenase - Methylcrotonyl-CoA carboxylase TRYPTOPHAN→acetyl-CoA Kynureninase PHENYLALANINE→tyrosine→acetoacetate (see below)
G	glycine→serine→pyruvate→TCA Serine hydroxymethyltransferase - Serine dehydratase alanine→pyruvate→TCA Alanine transaminase cysteine→pyruvate→TCA D-cysteine desulfhydrase threonine→pyruvate→TCA L-threonine dehydrogenase HISTIDINE→glutamate Histidine ammonia-lyase - Urocanate hydratase - Formiminotransferase cyclodeaminase proline→glutamate Proline oxidase - Pyrroline-5-carboxylate reductase - 1-Pyrroline-5-carboxylate dehydrogenase/ALDH4A1 - PYCR1 arginine→glutamate Ornithine aminotransferase - Ornithine decarboxylase - Agmatinase glutamate→alpha-ketoglutarate→TCA Glutamate dehydrogenase METHIONINE→propionyl-CoA Methionine adenosyltransferase - Adenosylhomocysteinase - (or MTR/Homocysteine methyltransferase) - Cystathionine beta synthase - Cystathionine gamma-lyase ISOLEUCINE/valine→propionyl-CoA Branched-chain alpha-keto acid dehydrogenase complex
NT	HISTIDINE→histamine Histidine decarboxylase tyrosine→dopamine→epinephrine Tyrosine hydroxylase - Aromatic L-amino acid decarboxylase - Dopamine beta hydroxylase - Phenylethanolamine N-methyltransferase - Catechol-O-methyl transferase glutamate→GABA Glutamate decarboxylase arginine→NO Nitric oxide synthase
Other	tyrosine→melanin Tyrosinase TRYPTOPHAN→serotonin→melatonin Tryptophan hydroxylase - Aromatic L-amino acid decarboxylase - Acetylserotonin O-methyltransferase cysteine+glutamate→glutathione Gamma-glutamylcysteine synthetase - Glutathione synthetase glycine→creatine Guanidinoacetate N-methyltransferase - Creatine kinase glutamate→glutamine Glutamine synthetase - Glutaminase collagen Prolyl hydroxylase - Lysyl hydroxylase
see also disorders, intermediates