Branched-chain alpha-keto acid dehydrogenase complex
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| 2-oxoisovalerate dehydrogenase (lipoamide)
| |
| Identifiers | |
| Symbol | OVD1A |
| Entrez | 5014 |
| HUGO | 8523 |
| Other data | |
| EC number | 1.2.4.4 |
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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. Examples of these include valine, isoleucine, and leucine.
Cofactors
This complex requires the following 5 cofactors:
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.[1]. Other mitochondrial autoantigens include pyruvate dehydrogenase and branched chain oxoglutarate dehydrogenase, which are antigens recognized by anti-mitochondrial antibodies.
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
Antigens: Autoantigens |
|---|
| Acetylcholine receptor - Actin - Apolipoprotein H - Branched-chain alpha-keto acid dehydrogenase complex - Cardiolipin - Centromere - Epidermal transglutaminase - gangliosides - Oxoglutarate dehydrogenase - Sp100 nuclear antigen - Pyruvate dehydrogenase - Thrombin - Tissue transglutaminase - Topoisomerase - Nucleoporins (Nucleoporin-62 - Nucleoporin-210) |
Aldehyde/oxo oxidoreductases (EC 1.2) | |
|---|---|
| 1.2.1 - NAD or NADP acceptor | Aldehyde dehydrogenase - Acetaldehyde dehydrogenase (ALDH2) - Glyceraldehyde 3-phosphate dehydrogenase - Long-chain-aldehyde dehydrogenase |
| 1.2.4 - disulfide acceptor | Oxoglutarate dehydrogenase - Pyruvate dehydrogenase - Branched-chain alpha-keto acid dehydrogenase complex |
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
