The branched-chain α-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.
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This complex requires the following 5 cofactors:
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.
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 inflammatory immune responses. Some of these inflammatory 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.
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