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glutaryl-Coenzyme A dehydrogenase
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| 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.
Pathology
A defect in this enzyme is associated with neurological condition glutaric acidemia type 1, which is often fatal[1].
References
- ^ The Effect of a Glu370Asp Mutation in Glutaryl-CoA Dehydrogenase on Proton Transfer to the Dienolate Intermediate
External links
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