Glutaryl-CoA dehydrogenase
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| glutaryl-CoA dehydrogenase (decarboxylating) | |||||||||
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ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
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Glutaryl-CoA dehydrogenase (GCDH) is an
transcript variants.[5]
Structure
GCDH is a tetramer with
water molecules, which gets displaced when the substrate binds to the enzyme. The binding pocket is also smaller than some of the other ACD binding pockets because it is responsible for the chain-length specificity of GCDH for alternate substrates.[6] The GCDH gene is mapped onto 19p13.2 and has an exon count of 15.[7]
Function
GCDH is mainly known for the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and carbon dioxide, which is common in the mitochondrial oxidation of lysine, tryptophan, and hydroxylysine. The way it completes this task is through a series of physical, chemical, and electron-transfer steps. It first binds glutaryl-CoA substrate to the oxidized form of the enzyme and abstracts the
dienolate anion, a proton, and CO2. The dienolate intermediate is protonated, resulting in crotonyl-CoA and a release of products from the active site. Finally, the 2e−-reduced form of FAD is oxidized to two 1e− steps by an external electron acceptor to complete the turnover.[8]
Clinical significance
monomers and/or dimers.[6]
Interactions
GCDH has been seen to interact with:
References
- ^ a b c GRCh38: Ensembl release 89: ENSG00000105607 – Ensembl, May 2017
- ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000003809 – Ensembl, May 2017
- ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
- ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
- ^ "GCDH glutaryl-CoA dehydrogenase [ Homo sapiens (human) ]". NCBI. Retrieved 6 August 2015.
- ^ PMID 15274622.
- ^ PMID 24973495.
- PMID 17176108.
External links
- Glutaryl-CoA+dehydrogenase at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
- PDBe-KB provides an overview of all the structure information available in the PDB for Human Glutaryl-CoA dehydrogenase, mitochondrial