Branched-chain alpha-keto acid dehydrogenase complex

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:
 * Thiamine diphosphate
 * FAD
 * NAD+
 * Lipoate
 * Coenzyme A

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. . Other mitochondrial autoantigens include pyruvate dehydrogenase and branched chain oxoglutarate dehydrogenase, which are antigens recognized by anti-mitochondrial antibodies.