Glutaryl-CoA dehydrogenase

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].

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

This biochemistry article is a stub. You can help Wikipedia by expanding it.

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

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)

glycine→serine→	Serine hydroxymethyltransferase - Serine dehydratase

alanine→	Alanine transaminase

cysteine→	D-cysteine desulfhydrase

threonine→	L-threonine dehydrogenase


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


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