› 1x80

X-ray diffraction
2Å resolution

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

Released:
DOI: 10.2210/pdb1x80/pdb
PDB entry 1x80 coloured by chain, front view.
PDB entry 1x80 coloured by chain, side view.
PDB entry 1x80 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: 45.65 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
4 bound ligands:
Ligand K 2 x K
Ligand MN 1 x MN
Ligand CL 1 x CL
Ligand GOL 1 x GOL
1 modified residue:
Ligand SEP 1 x SEP

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

Quick links

Citations

8 review citations

Lipoic acid metabolism and mitochondrial redox regulation.
Solmonson et al. (2018)
7 more

1 mention without citation

Nuclear inelastic scattering and Mössbauer spectroscopy as local probes for ligand binding modes and electronic properties in proteins: vibrational behavior of a ferriheme center inside a β-barrel protein.
Moeser et al. (2012)