2-oxoisovalerate dehydrogenase
From Proteopedia
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== Function == | == Function == | ||
| - | '''2-oxoisovalerate dehydrogenase''' (OIVD) or '''branched-chain α-keto acid decarboxylase''' is part of a multienzyme complex metabolizing pyruvate, 2-oxogluterate and branched-chain 2-oxo acids. The multienzyme complex ('''BCKDC''') contains three catalytic components: OIVD, lipoamide acyltransferase and lipoamide dehydrogenase, a kinase and a phosphatase. The active site of OIVD contains the cofactor thiamin diphosphate (ThDP)<ref>PMID:10426958</ref>. | + | '''2-oxoisovalerate dehydrogenase''' (OIVD) or '''branched-chain α-keto acid decarboxylase''' or '''branched-chain α-keto acid dehedrogenase''' is part of a multienzyme complex metabolizing pyruvate, 2-oxogluterate and branched-chain 2-oxo acids. The multienzyme complex ('''BCKDC''') contains three catalytic components: OIVD, lipoamide acyltransferase and lipoamide dehydrogenase, a kinase and a phosphatase. The active site of OIVD contains the cofactor thiamin diphosphate (ThDP)<ref>PMID:10426958</ref>. |
== Disease == | == Disease == | ||
Revision as of 08:49, 25 February 2021
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References
- ↑ Aevarsson A, Seger K, Turley S, Sokatch JR, Hol WG. Crystal structure of 2-oxoisovalerate and dehydrogenase and the architecture of 2-oxo acid dehydrogenase multienzyme complexes. Nat Struct Biol. 1999 Aug;6(8):785-92. PMID:10426958 doi:10.1038/11563
- ↑ Wang YP, Qi ML, Li TT, Zhao YJ. Two novel mutations in the BCKDHB gene (R170H, Q346R) cause the classic form of maple syrup urine disease (MSUD). Gene. 2012 Apr 25;498(1):112-5. doi: 10.1016/j.gene.2012.01.082. Epub 2012 Feb 3. PMID:22326532 doi:10.1016/j.gene.2012.01.082
- ↑ Wynn RM, Kato M, Machius M, Chuang JL, Li J, Tomchick DR, Chuang DT. Molecular mechanism for regulation of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase complex by phosphorylation. Structure. 2004 Dec;12(12):2185-96. PMID:15576032 doi:10.1016/j.str.2004.09.013
