Branched-chain alpha-keto acid dehydrogenase complex
|2-oxoisovalerate dehydrogenase (lipoamide)|
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.
This complex requires the following 5 cofactors:
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.. Other mitochondrial autoantigens include pyruvate dehydrogenase and branched chain oxoglutarate dehydrogenase, which are antigens recognized by anti-mitochondrial antibodies.
- Branched+Chain+Ketoacid+Dehydrogenase at the US National Library of Medicine Medical Subject Headings (MeSH)
- EC 18.104.22.168