Glutaryl-CoA dehydrogenase

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glutaryl-Coenzyme A dehydrogenase
Symbol GCDH
Entrez 2639
HUGO 4189
OMIM 608801
RefSeq NM_013976
UniProt Q92947
Other data
EC number
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.


A defect in this enzyme is associated with neurological condition glutaric acidemia type 1, which is often fatal.[1] It has been found that mutations in this gene cause a progressive form of early-onset generalized dystonia.[2]


  1. ^ The Effect of a Glu370Asp Mutation in Glutaryl-CoA Dehydrogenase on Proton Transfer to the Dienolate Intermediate
  2. ^ Marti-Masso, JF; Ruiz-Martínez, J, Makarov, V, de Munain, AL, Gorostidi, A, Bergareche, A, Yoon, S, Buxbaum, JD, Paisán-Ruiz, C (Sep 13, 2011). "Exome sequencing identifies GCDH (glutaryl-CoA dehydrogenase) mutations as a cause of a progressive form of early-onset generalized dystonia.". Human Genetics 131 (3): 435–42. doi:10.1007/s00439-011-1086-6. PMID 21912879. 

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