2-oxoisovalerate dehydrogenase

< StructureSection load='1x80' size='340' side='right' caption='Human 2-oxoisovalerate dehydrogenase α (deepskyblue and cyan) and β (coral and orangered) subunit with phosphorylated Ser residue complex with thiamine diphosphate, glycerol, K+ ion (purple), Cl- ion (green) and Mn+2 ion (PDB code [[1x80]])' scene='80/800706/Cv/2'>

== 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>.

== Disease ==

Mutations in OIVD result in maple syrup urine disease characterized by mental and physical retardation and feeding problems<ref>PMID:22326532</ref>.

== Structural highlights ==

<scene name='80/800706/Cv/3'>Human 2-oxoisovalerate dehydrogenase α and β subunit with phosphorylated Ser residue complex with thiamine diphosphate, glycerol, K+ ion, Cl- ion, and Mn+2 ion</scene>. The structure of the phosphorylated human OIVD shows <scene name='80/800706/Cv/5'>the cofactor ThDP interacting with residues from both α and β subunits</scene<ref>PMID:15576032</ref>.

</StructureSection>

==3D structures of 2-oxoisovalerate dehydrogenase==

Updated on {{REVISIONDAY2}}-{{MONTHNAME|{{REVISIONMONTH}}}}-{{REVISIONYEAR}}

[[2bp7]] - OIVD α+β subunits - ''Pseudomonas putida''<br />

[[1v1r]] - hOIVD α+β subunits - human<br />

[[1u5b]], [[1x80]], [[1ols]] - hOIVD α+β subunits + ThDP <br />

[[1x7x]], [[1x7w]], [[1x7y]], [[1x7z]], [[2j9f]], [[1olu]], [[2bfb]], [[2bfd]], [[2bfe]], [[1v11]] - hOIVD α (mutant)+ β subunits + ThDP derivative<br />

[[1olx]] - hOIVD α+β (mutant) subunits + ThDP<br />

[[1wci]] - hOIVD α+β subunits + thiamine derivative<br />

[[2bfe]] - hOIVD α+β subunits + pyrimidine derivative<br />

[[2bfc]] - hOIVD α (mutant)+ β subunits + pyrimidine derivative<br />

[[1v16]] - hOIVD α+β subunits + ThDP + benzamidine<br />

[[1v1m]] - hOIVD α (mutant)+ β subunits + ThDP + benzamidine<br />

[[2bew]], [[2beu]], [[2bev]] - hOIVD α+β subunits + ThDP derivative + peptide<br />

== References ==

<references/>

[[Category:Topic Page]]