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1X7Z

Crystal structure of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase

Entity
Entity IDChain IDDescriptionTypeChain lengthFormula weightNumber of moleculesDB Name (Accession)Biological sourceDescriptive keywords
1A
(A)
2-oxoisovalerate dehydrogenase alpha subunitpolymer40045599.11UniProt (P12694)
Pfam (PF00676)
Homo sapiens (human)Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain, BCKDH E1-alpha, BCKDE1A
2B
(B)
2-oxoisovalerate dehydrogenase beta subunitpolymer34237902.31UniProt (P21953)
Pfam (PF02779)
Pfam (PF02780)
Homo sapiens (human)Branched-chain alpha-keto acid dehydrogenase E1 component beta chain, BCKDH E1-beta
3C, G
(A, B)
POTASSIUM IONnon-polymer39.12Chemie (K)
4D
(A)
MANGANESE (II) IONnon-polymer54.91Chemie (MN)
5E
(A)
CHLORIDE IONnon-polymer35.51Chemie (CL)
6F
(A)
THIAMINE DIPHOSPHATEnon-polymer425.31Chemie (TPP)
7H
(B)
GLYCEROLnon-polymer92.11Chemie (GOL)
8I, J
(A, B)
waterwater18.0636Chemie (HOH)
Sequence modifications
A: 1 - 400 (UniProt: P12694)
PDBExternal DatabaseDetails
Asp 292Ser 337engineered mutation
Sequence viewer
Contents of the asymmetric unit
PolymersNumber of chains2
Total formula weight83501.4
Non-Polymers*Number of molecules6
Total formula weight686.0
All*Total formula weight84187.4
*Water molecules are not included.

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PDB entries from 2024-10-30

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