› 2cw6

X-ray diffraction
2.1Å resolution

Crystal Structure of Human HMG-CoA Lyase: Insights into Catalysis and the Molecular Basis for Hydroxymethylglutaric Aciduria

Released:
DOI: 10.2210/pdb2cw6/pdb
PDB entry 2cw6 coloured by chain, front view.
PDB entry 2cw6 coloured by chain, side view.
PDB entry 2cw6 coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Crystal structure of human 3-hydroxy-3-methylglutaryl-CoA Lyase: insights into catalysis and the molecular basis for hydroxymethylglutaric aciduria.
Fu Z, Runquist JA, Forouhar F, Hussain M, Hunt JF, Miziorko HM, Kim JJ
J Biol Chem 281 7526-32 (2006)
PMID: 16330550

Function and Biology Details

Reaction catalysed: 
(S)-3-hydroxy-3-methylglutaryl-CoA = acetyl-CoA + acetoacetate
Biochemical function:
Biological process:
Cellular component:
Sequence domains:

Structure analysis Details

Assemblies composition:
homo dimer (preferred)
homo dodecamer
homo hexamer
Assembly name:
PDBe Complex ID:
PDB-CPX-153052 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecule:
Hydroxymethylglutaryl-CoA lyase, mitochondrial Chains: A, B, C, D, E, F
Molecule details ›
Chains: A, B, C, D, E, F
Length: 298 amino acids
Theoretical weight: 31.58 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli
UniProt:
  • Canonical: P35914 (Residues: 28-325; Coverage: 92%)
Gene name: HMGCL
Sequence domains: HMGL-like
Structure domains: Aldolase class I

Ligands and Environments

2 bound ligands:
Ligand MG 6 x MG
Ligand 3HG 1 x 3HG
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 14-BM-C
Spacegroup: C2
Unit cell:
a: 196.99Å b: 117.08Å c: 86.83Å
α: 90° β: 112.5° γ: 90°
R-values:
R R work R free
0.226 0.226 0.265
Expression system: Escherichia coli

Quick links

Citations

2 review citations

Enzymes involved in branched-chain amino acid metabolism in humans.
Adeva-Andany et al. (2017)
1 more

7 mentions without citation

Evaluation of the template-based modeling in CASP12.
Kryshtafovych et al. (2018)
6 more