› 5mgw

X-ray diffraction
1.46Å resolution

Kinetic and Structural Changes in HsmtPheRS, Induced by Pathogenic Mutations in Human FARS2

Released:
DOI: 10.2210/pdb5mgw/pdb
PDB entry 5mgw coloured by chain, front view.
PDB entry 5mgw coloured by chain, side view.
PDB entry 5mgw coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Kinetic and structural changes in HsmtPheRS, induced by pathogenic mutations in human FARS2.
Kartvelishvili E, Tworowski D, Vernon H, Moor N, Wang J, Wong LJ, Chrzanowska-Lightowlers Z, Safro M
Protein Sci 26 1505-1516 (2017)
PMID: 28419689

Function and Biology Details

Reaction catalysed: 
ATP + L-phenylalanine + tRNA(Phe) = AMP + diphosphate + L-phenylalanyl-tRNA(Phe)
Biochemical function:
Biological process:
Cellular component:
Sequence domains:

Structure analysis Details

Assembly composition:
monomeric (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-131898 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecule:
Phenylalanine--tRNA ligase, mitochondrial Chain: A
Molecule details ›
Chain: A
Length: 406 amino acids
Theoretical weight: 47.49 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli
UniProt:
  • Canonical: O95363 (Residues: 46-451; Coverage: 90%)
Gene names: FARS1, FARS2, HSPC320
Sequence domains:
Structure domains:

Ligands and Environments

1 bound ligand:
Ligand PHE 1 x PHE
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: ESRF BEAMLINE ID23-1
Spacegroup: P212121
Unit cell:
a: 53.355Å b: 90.117Å c: 99.081Å
α: 90° β: 90° γ: 90°
R-values:
R R work R free
0.186 0.185 0.205
Expression system: Escherichia coli

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Citations

1 review citation

When a common biological role does not imply common disease outcomes: Disparate pathology linked to human mitochondrial aminoacyl-tRNA synthetases.
González-Serrano et al. (2019)
5 other articles

2 mentions without citation

Kinetic and structural changes in HsmtPheRS, induced by pathogenic mutations in human FARS2.
Kartvelishvili et al. (2017)
1 more