› 1x7y

X-ray diffraction
1.57Å resolution

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

Released:
DOI: 10.2210/pdb1x7y/pdb
PDB entry 1x7y coloured by chain, front view.
PDB entry 1x7y coloured by chain, side view.
PDB entry 1x7y coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Molecular mechanism for regulation of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase complex by phosphorylation.
Wynn RM, Kato M, Machius M, Chuang JL, Li J, Tomchick DR, Chuang DT
Structure 12 2185-96 (2004)
PMID: 15576032

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)
Biochemical function:
Biological process:
Cellular component:
Sequence domains:
Structure domains:

Structure analysis Details

Assembly composition:
hetero tetramer (preferred)
Assembly name:
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: 45.6 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
4 bound ligands:
Ligand K 2 x K
Ligand MN 1 x MN
Ligand CL 1 x CL
Ligand GOL 1 x GOL
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 19-ID
Spacegroup: P3121
Unit cell:
a: 145.672Å b: 145.672Å c: 69.236Å
α: 90° β: 90° γ: 120°
R-values:
R R work R free
0.15 0.15 0.167
Expression system: Escherichia coli BL21

Quick links

Citations

7 review citations

Mitochondrial modulation: reversible phosphorylation takes center stage?
Pagliarini et al. (2006)
6 more

3 mentions without citation

Protein beta-sheet nucleation is driven by local modular formation.
Wathen et al. (2010)
2 more