Branched-chain alpha-keto acid dehydrogenase complex 3360546 211344240 2008-05-09T21:45:11Z Arcadian 104523 nav {{protein |Name=2-oxoisovalerate dehydrogenase (lipoamide) |caption= |image= |width= |HGNCid=8523 |Symbol=OVD1A |AltSymbols= |EntrezGene=5014 |OMIM= |RefSeq= |UniProt= |PDB= |ECnumber=1.2.4.4 |Chromosome= |Arm= |Band= |LocusSupplementaryData= }} The '''branched-chain α-keto acid dehydrogenase complex''' is a combination of [[enzyme]]s 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]] *[[Nicotinamide adenine dinucleotide|NAD<sup>+</sup>]] *[[Lipoic acid|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 [[enzyme inhibitor|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]].<ref name="pmid17657817">{{cite journal | author = Leung PS, Rossaro L, Davis PA, ''et al'' | title = Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis | journal = | volume = | issue = | pages = | year = 2007 | pmid = 17657817 | doi = 10.1002/hep.21828}}</ref>. Other mitochondrial autoantigens include [[pyruvate dehydrogenase]] and branched chain [[oxoglutarate dehydrogenase]], which are antigens recognized by [[anti-mitochondrial antibodies]]. ==References== {{Reflist}} ==External links== * {{MeshName|Branched+Chain+Ketoacid+Dehydrogenase}} * {{EC number|1.2.4.4}} {{Autoantigens}} {{Aldehyde/Oxo oxidoreductases}} {{Amino acid metabolism enzymes}} [[Category:autoantigens]] {{oxidoreductase-stub}}