Glutaryl-CoA dehydrogenase

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glutaryl-Coenzyme A dehydrogenase
Identifiers
Symbol	GCDH
Entrez	2639
HUGO	4189
OMIM	608801
RefSeq	NM_013976
UniProt	Q92947
Other data
EC number	1.3.99.7
Locus	Chr. 19 p13.2

Glutaryl-CoA dehydrogenase is an enzyme acting upon glutaryl-coenzyme A, creating crotonyl-coenzyme A.

It plays a role in the metabolism of lysine, hydroxylysine and tryptophan.

[edit] Pathology

A defect in this enzyme is associated with neurological condition glutaric acidemia type 1, which is often fatal^[1].

[edit] References

1. ^ The Effect of a Glu370Asp Mutation in Glutaryl-CoA Dehydrogenase on Proton Transfer to the Dienolate Intermediate

[edit] External links

• MeSH Glutaryl-CoA+dehydrogenase

v • d • e Oxidoreductases: CH-CH oxidoreductases (EC 1.3) 1.3.1. NAD/NADP acceptor Enoyl-acyl carrier protein reductase/Enoyl ACP reductase - 7-Dehydrocholesterol reductase - Biliverdin reductase - 2,4 Dienoyl-CoA reductase 1.3.3. Oxygen acceptor Dihydroorotate dehydrogenase - Coproporphyrinogen III oxidase - Protoporphyrinogen oxidase - 1.3.5. Quinone Succinate dehydrogenase 1.3.99. Other acceptors Fumarate reductase - Butyryl CoA dehydrogenase - Acyl CoA dehydrogenase - 5-alpha reductase - Glutaryl-CoA dehydrogenase - Isovaleryl coenzyme A dehydrogenase

v • d • e Metabolism: amino acid metabolism - synthesis and catabolism enzymes (essential in CAPS) K→acetyl-CoA LYSINE→ Saccharopine dehydrogenase - Glutaryl-CoA dehydrogenase LEUCINE→ Branched chain aminotransferase - Branched-chain alpha-keto acid dehydrogenase complex - Isovaleryl coenzyme A dehydrogenase - Methylcrotonyl-CoA carboxylase - Methylglutaconyl-CoA hydratase - 3-hydroxy-3-methylglutaryl-CoA lyase TRYPTOPHAN→ Indoleamine 2,3-dioxygenase/Tryptophan 2,3-dioxygenase - Arylformamidase - Kynureninase - 3-hydroxyanthranilate oxidase - Aminocarboxymuconate-semialdehyde decarboxylase - Aminomuconate-semialdehyde dehydrogenase PHENYLALANINE→tyrosine→ (see below) G→pyruvate glycine→serine→ Serine hydroxymethyltransferase - Serine dehydratase alanine→ Alanine transaminase cysteine→ D-cysteine desulfhydrase threonine→ L-threonine dehydrogenase G→glutamate 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 G→propionyl-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→ Methionine adenosyltransferase - Adenosylhomocysteinase - Cystathionine beta synthase - Cystathionine gamma-lyase (methionine synthesis: MTR/Homocysteine methyltransferase) THREONINE→ Threonine aldolase →succinyl-CoA→TCA Propionyl-CoA carboxylase - Methylmalonyl-CoA mutase G→fumarate PHENYLALANINE→tyrosine→ Phenylalanine hydroxylase - Tyrosine aminotransferase - 4-Hydroxyphenylpyruvate dioxygenase - Homogentisate 1,2-dioxygenase - Fumarylacetoacetate hydrolase G→oxaloacetate asparagine→aspartate→ Asparaginase/Asparagine synthetase - Aspartate transaminase Other tyrosine→melanin Tyrosinase cysteine+glutamate→glutathione Gamma-glutamylcysteine synthetase - Glutathione synthetase - Gamma-glutamyl transpeptidase glycine→creatine Guanidinoacetate N-methyltransferase - Creatine kinase glutamate→glutamine Glutamine synthetase - Glutaminase proline+lysine→collagen Prolyl hydroxylase - Lysyl hydroxylase see also disorders, intermediates