2-oxoisovalerate dehydrogenase

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<StructureSection load='1x80' size='340' side='right' caption='Caption for this structure' scene=''>
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<StructureSection load='1x80' size='340' side='right' caption='Human 2-oxoisovalerate dehydrogenase α (grey) and β (green) subunit with phosphorylated Ser residue complex with thiamine diphosphate, glycerol, K+ ion (purple), Cl- ion (green) and Mn+2 ion (PDB code [[1x80]])' scene=''>
== Function ==
== Function ==
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'''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 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>.
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'''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>.
== Disease ==
== Disease ==

Revision as of 09:48, 6 November 2018

Human 2-oxoisovalerate dehydrogenase α (grey) and β (green) subunit with phosphorylated Ser residue complex with thiamine diphosphate, glycerol, K+ ion (purple), Cl- ion (green) and Mn+2 ion (PDB code 1x80)

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3D structures of 2-oxoisovalerate dehydrogenase

Updated on 06-November-2018

2bp7 - OIVD α+β subunits - Pseudomonas putida
1v1r - hOIVD α+β subunits - human
1u5b, 1x80, 1ols - hOIVD α+β subunits + ThDP
1x7x, 1x7w, 1x7y, 1x7z, 2j9f, 1olu, 2bfb, 2bfd, 2bfe, 1v11 - hOIVD α (mutant)+ β subunits + ThDP derivative
1olx - hOIVD α+β (mutant) subunits + ThDP
1wci - hOIVD α+β subunits + thiamine derivative
2bfe - hOIVD α+β subunits + pyrimidine derivative
2bfc - hOIVD α (mutant)+ β subunits + pyrimidine derivative
1v16 - hOIVD α+β subunits + ThDP + benzamidine
1v1m - hOIVD α (mutant)+ β subunits + ThDP + benzamidine
2bew, 2beu, 2bev - hOIVD α+β subunits + ThDP derivative + peptide

References

  1. ↑ 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
  2. ↑ 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

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