Methionine–tRNA ligase, mitochondrial

Methionine–tRNA ligase, mitochondrial, also called methionyl-tRNA synthetase 2, is an enzyme that in humans is encoded by the MARS2 gene. This enzyme functions as a methionine–tRNA ligase, which means it binds the amino acid methionine to its corresponding transfer RNA (tRNAMet), which is part of RNA-to-protein translation.

Medical Significance Pathogenic mutations in MARS2 are associated with problems with muscle coordination, and in particular a type of ataxia called autosomal recessive spastic ataxia-3 (SPAX3).

See also Aminoacyl tRNA synthetase

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