Branched-chain alpha-keto acid dehydrogenase complex
3360546
211344240
2008-05-09T21:45:11Z
Arcadian
104523
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{{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}}