Health Dictionary
Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Branched-chain alpha-keto acid dehydrogenase complex
Introduction
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
Cofactors
This complex requires the following 5 cofactors:
Similar enzymes
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
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
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
External links
- Branched+Chain+Ketoacid+Dehydrogenase at the US National Library of Medicine Medical Subject Headings (MeSH)
- EC 1.2.4.4
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]