› 5i35

X-ray diffraction
2.3Å resolution

Structure of the Human mitochondrial kinase COQ8A R611K with AMPPNP (Cerebellar Ataxia and Ubiquinone Deficiency Through Loss of Unorthodox Kinase Activity)

Released:
DOI: 10.2210/pdb5i35/pdb
PDB entry 5i35 coloured by chain, front view.
PDB entry 5i35 coloured by chain, side view.
PDB entry 5i35 coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Cerebellar Ataxia and Coenzyme Q Deficiency through Loss of Unorthodox Kinase Activity.
Stefely JA, Licitra F, Laredj L, Reidenbach AG, Kemmerer ZA, Grangeray A, Jaeg-Ehret T, Minogue CE, Ulbrich A, Hutchins PD, Wilkerson EM, Ruan Z, Aydin D, Hebert AS, Guo X, Freiberger EC, Reutenauer L, Jochem A, Chergova M, Johnson IE, Lohman DC, Rush MJP, Kwiecien NW, Singh PK, Schlagowski AI, Floyd BJ, Forsman U, Sindelar PJ, Westphall MS, Pierrel F, Zoll J, Dal Peraro M, Kannan N, Bingman CA, Coon JJ, Isope P, Puccio H, Pagliarini DJ
Mol Cell 63 608-620 (2016)
PMID: 27499294

Function and Biology Details

Biochemical function:
  • not assigned
Biological process:
  • not assigned
Cellular component:
  • not assigned
Sequence domains:

Structure analysis Details

Assembly composition:
monomeric (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-185573 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecule:
Atypical kinase COQ8A, mitochondrial Chain: A
Molecule details ›
Chain: A
Length: 393 amino acids
Theoretical weight: 45.92 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli
UniProt:
  • Canonical: Q8NI60 (Residues: 256-647; Coverage: 61%)
Gene names: ADCK3, CABC1, COQ8A, PP265
Sequence domains: ABC1 atypical kinase-like domain

Ligands and Environments

1 bound ligand:
Ligand ANP 1 x ANP
1 modified residue:
Ligand MSE 15 x MSE

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 23-ID-B
Spacegroup: C2
Unit cell:
a: 150.05Å b: 59.12Å c: 51Å
α: 90° β: 97.69° γ: 90°
R-values:
R R work R free
0.19 0.183 0.259
Expression system: Escherichia coli

Quick links

Citations

21 review citations

Live and let die: insights into pseudoenzyme mechanisms from structure.
Murphy et al. (2017)
20 more

8 mentions without citation

Biochemistry of Mitochondrial Coenzyme Q Biosynthesis.
Stefely et al. (2017)
7 more