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HUMAN BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE

X-ray diffraction
2.7Å resolution
PDB entry 1dtw coloured by chain, front view.
PDB entry 1dtw coloured by chain, side view.
PDB entry 1dtw coloured by chain, top view.
Source organism: Homo sapiens
Assembly composition:
hetero tetramer (preferred)
Entry contents: 2 distinct polypeptide molecules

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Assemblies

Assembly 1 (preferred)
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Assembly Name: 2-oxoisovalerate dehydrogenase mitochondrial
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Multimeric state: hetero tetramer
Accessible surface area: 42895.65 Å2
Buried surface area: 26103.05 Å2
Dissociation area: 8,363.18 Å2
Dissociation energy (ΔGdiss): 128.47 kcal/mol
Dissociation entropy (TΔSdiss): 17.31 kcal/mol
Symmetry number: 2
PDBe Complex ID: PDB-CPX-146300

Macromolecules

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Chain: A
Length: 400 amino acids
Theoretical weight: 45.57 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli
UniProt:
  • Canonical: P12694 (Residues: 46-445; Coverage: 90%)
Gene name: BCKDHA
Pfam: Dehydrogenase E1 component
InterPro:
CATH: Rossmann fold
SCOP: Branched-chain alpha-keto acid dehydrogenase PP module
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial in PDB entry 1dtw, assembly 1, front view.
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial in PDB entry 1dtw, assembly 1, side view.
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial in PDB entry 1dtw, assembly 1, top view.
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2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Chain: B
Length: 342 amino acids
Theoretical weight: 37.9 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli
UniProt:
  • Canonical: P21953 (Residues: 51-392; Coverage: 87%)
Gene name: BCKDHB
Pfam:
InterPro:
CATH:
SCOP:
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial in PDB entry 1dtw, assembly 1, front view.
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial in PDB entry 1dtw, assembly 1, side view.
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial in PDB entry 1dtw, assembly 1, top view.
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