Branched-chain alpha-keto acid dehydrogenase complex
| 2-oxoisovalerate dehydrogenase (lipoamide) | |
|---|---|
| Identifiers | |
| Symbol | OVD1A |
| Entrez | 5014 |
| HUGO | 8523 |
| Other data | |
| EC number | 1.2.4.4 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
The branched-chain alpha-keto acid dehydrogenase complex is a combination of enzymes responsible for the degradation of the branched chain amino acids. Examples of these include valine, isoleucine, and leucine.
Cofactors
This complex requires the following 5 cofactors:
Similar enzymes
This complex is also analogous to the alpha-ketoglutarate dehydrogenase complex in the citric acid cycle and the pyruvate dehydrogenase complex creating acetyl-CoA prior to the citric acid cycle.
Pathology
A deficiency in any of the enzymes of this complex as well as an inhibition of the complex as a whole is responsible for maple syrup urine disease.
This enzyme is an autoantigen recognized in primary biliary cirrhosis, a form of acute liver failure. These antibodies appear to recognize oxidized protein that has resulted from inflamatory immune responses. Some of these inflamatory responses are explained by gluten sensitivity.[1]. Other mitochondrial autoantigens include pyruvate dehydrogenase and branched chain oxoglutarate dehydrogenase, which are antigens recognized by anti-mitochondrial antibodies.
References
- ↑ Leung PS, Rossaro L, Davis PA; et al. (2007). "Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis". doi:10.1002/hep.21828. PMID 17657817.
External links
- Branched+Chain+Ketoacid+Dehydrogenase at the US National Library of Medicine Medical Subject Headings (MeSH)
- EC 1.2.4.4