1x80

X-ray diffraction
2Å resolution

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

Released:

Function and Biology Details

Reaction catalysed:
3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO(2)

Structure analysis Details

Assembly composition:
hetero tetramer (preferred)
PDBe Complex ID:
PDB-CPX-146300 (preferred)
Entry contents:
2 distinct polypeptide molecules
Macromolecules (2 distinct):
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial Chain: A
Molecule details ›
Chain: A
Length: 400 amino acids
Theoretical weight: 45.65 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli BL21
UniProt:
  • Canonical: P12694 (Residues: 46-445; Coverage: 90%)
Gene name: BCKDHA
Sequence domains: Dehydrogenase E1 component
Structure domains: Rossmann fold
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial Chain: B
Molecule details ›
Chain: B
Length: 342 amino acids
Theoretical weight: 37.9 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli BL21
UniProt:
  • Canonical: P21953 (Residues: 51-392; Coverage: 87%)
Gene name: BCKDHB
Sequence domains:
Structure domains:

Ligands and Environments


Cofactor: Ligand TPP 1 x TPP
1 modified residue:

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 19-ID
Spacegroup: P3121
Unit cell:
a: 144.403Å b: 144.403Å c: 69.009Å
α: 90° β: 90° γ: 120°
R-values:
R R work R free
0.161 0.16 0.193
Expression system: Escherichia coli BL21