Branched-chain alpha-keto acid dehydrogenase complex

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The branched-chain alpha-keto acid dehydrogenase complex is a combination of enzymes responsible for the degradation of the branched chain amino acids (valine, leucine, and isoleucine). Examples of these include valine, isoleucine, and leucine. 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 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.


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