› 1rx0

X-ray diffraction
1.77Å resolution

Crystal structure of isobutyryl-CoA dehydrogenase complexed with substrate/ligand.

Released:
DOI: 10.2210/pdb1rx0/pdb
PDB entry 1rx0 coloured by chain, front view.
PDB entry 1rx0 coloured by chain, side view.
PDB entry 1rx0 coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Structures of isobutyryl-CoA dehydrogenase and enzyme-product complex: comparison with isovaleryl- and short-chain acyl-CoA dehydrogenases.
Battaile KP, Nguyen TV, Vockley J, Kim JJ
J Biol Chem 279 16526-34 (2004)
PMID: 14752098

Function and Biology Details

Reaction catalysed: 
2-methylbutanoyl-CoA + electron-transfer flavoprotein = (E)-2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H(+)
Biochemical function:
Biological process:
Cellular component:
Sequence domains:
Structure domains:

Structure analysis Details

Assembly composition:
homo tetramer (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-193860 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecule:
Isobutyryl-CoA dehydrogenase, mitochondrial Chains: A, B, C, D
Molecule details ›
Chains: A, B, C, D
Length: 393 amino acids
Theoretical weight: 42.75 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli BL21
UniProt:
  • Canonical: Q9UKU7 (Residues: 24-415; Coverage: 95%)
Gene names: ACAD8, ARC42, IBD
Sequence domains:
Structure domains:

Ligands and Environments


Cofactor: Ligand FAD 4 x FAD
3 bound ligands:
Ligand EDO 11 x EDO
Ligand ACY 7 x ACY
Ligand 2MC 2 x 2MC
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 14-BM-C
Spacegroup: P212121
Unit cell:
a: 97.204Å b: 134.343Å c: 189.641Å
α: 90° β: 90° γ: 90°
R-values:
R R work R free
0.181 0.179 0.192
Expression system: Escherichia coli BL21

Quick links

Citations

4 review citations

The diverse roles of flavin coenzymes--nature's most versatile thespians.
Mansoorabadi et al. (2007)
3 more

11 mentions without citation

Phenotype, genotype and long-term prognosis of 40 Chinese patients with isobutyryl-CoA dehydrogenase deficiency and a review of variant spectra in ACAD8.
Feng et al. (2021)
10 more