Urocanate hydratase: Information from Answers.com

Urocanate hydratase (also known as urocanase) is an enzyme involved in the conversion of urocanate and imidazol-4-one-5-propionic acid. Inherited deficiency of urocanate hydratase leads to elevated levels of urocanic acid in the urine, a condition known as urocanic aciduria.

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MeSH Urocanate+Hydratase
EC 4.2.1.49

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v • d • e Carbon-oxygen lyases (EC 4.2) (primarily dehydratases)

4.2.1: Hydro-Lyases	Carbonic anhydrase - Fumarase - Aconitase - Enolase (Alpha, Enolase 2) - Enoyl-CoA hydratase/3-Hydroxyacyl ACP dehydrase - Methylglutaconyl-CoA hydratase - Tryptophan synthase - Cystathionine beta synthase - Porphobilinogen synthase - 3-isopropylmalate dehydratase - Urocanate hydratase - Uroporphyrinogen III synthase - Nitrile hydratase

4.2.2: Acting on polysaccharides	Hyaluronate lyase

4.2.3: Acting on phosphates	Threonine synthase

4.2.99: Other	Carboxymethyloxysuccinate lyase

Metabolism: amino acid metabolism · synthesis and catabolism enzymes (essential in CAPS)

K→acetyl-CoA

G→pyruvate
→citrate

glycine→serine→	Serine hydroxymethyltransferase · Serine dehydratase glycine→creatine: Guanidinoacetate N-methyltransferase · Creatine kinase

alanine→	Alanine transaminase

cysteine→	D-cysteine desulfhydrase

threonine→	L-threonine dehydrogenase

G→glutamate→
α-ketoglutarate


HISTIDINE→	Histidine ammonia-lyase · Urocanate hydratase · Formiminotransferase cyclodeaminase

proline→	Proline oxidase · Pyrroline-5-carboxylate reductase · 1-Pyrroline-5-carboxylate dehydrogenase/ALDH4A1 · PYCR1

arginine→	Ornithine aminotransferase · Ornithine decarboxylase · Agmatinase

→alpha-ketoglutarate→TCA	Glutamate dehydrogenase

Other	cysteine+glutamate→glutathione: Gamma-glutamylcysteine synthetase · Glutathione synthetase · Gamma-glutamyl transpeptidase glutamate→glutamine: Glutamine synthetase · Glutaminase

G→propionyl-CoA→
succinyl-CoA

VALINE→	Branched chain aminotransferase · Branched-chain alpha-keto acid dehydrogenase complex · Enoyl-CoA hydratase · 3-hydroxyisobutyryl-CoA hydrolase · 3-hydroxyisobutyrate dehydrogenase · Methylmalonate semialdehyde dehydrogenase

ISOLEUCINE→	Branched chain aminotransferase · Branched-chain alpha-keto acid dehydrogenase complex · 3-hydroxy-2-methylbutyryl-CoA dehydrogenase

METHIONINE→	generation of homocysteine: Methionine adenosyltransferase · Adenosylhomocysteinase regeneration of methionine: MTR/Homocysteine methyltransferase · Betaine-homocysteine methyltransferase conversion to cysteine: Cystathionine beta synthase · Cystathionine gamma-lyase

THREONINE→	Threonine aldolase

→succinyl-CoA→TCA	Propionyl-CoA carboxylase · Methylmalonyl CoA epimerase · Methylmalonyl-CoA mutase

G→fumarate


PHENYLALANINE→tyrosine→	Phenylalanine hydroxylase · Tyrosine aminotransferase · 4-Hydroxyphenylpyruvate dioxygenase · Homogentisate 1,2-dioxygenase · Fumarylacetoacetate hydrolase tyrosine→melanin: Tyrosinase

G→oxaloacetate


asparagine→aspartate→	Asparaginase/Asparagine synthetase · Aspartate transaminase

see also disorders, intermediates

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	3-isopropylmalate dehydratase
	Dehydratase
	Methylglutaconyl-CoA hydratase