This gene encodes a mitochondrial aminoacyl-tRNA synthetase, which catalyzes the attachment of valine to tRNA(Val) for mitochondrial translation. Mutations in this gene cause combined oxidative phosphorylation deficiency-20, and are also associated with early-onset mitochondrial encephalopathies. Alternative splicing of this gene results in multiple transcript variants.
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Protein Aliases: valine tRNA ligase 2, mitochondrial (putative); Valine--tRNA ligase, mitochondrial; ValRS; valyl trna synthetase; Valyl-tRNA synthetase; valyl-tRNA synthetase 2, mitochondrial (putative); valyl-tRNA synthetase like; valyl-tRNA synthetase, mitochondrial; Valyl-tRNA synthetase-like
Gene Aliases: 1190004I24Rik; COXPD20; KIAA1885; mKIAA1885; VALRS; VARS2; VARS2L; VARSL