Mixing and matching mitochondrial aminoacyl synthetases and their tRNAs: a new way to treat respiratory chain disorders?

Mutations in mitochondrial DNA are an important cause of human disease and from a therapeutic standpoint, these disorders are currently untreatable. New studies now show that a non-cognate mitochondrial aminoacyl tRNA synthetase can overcome the respiratory defect caused by an mt-tRNA mutation and that the isolated carboxy-terminal domain of human mt-leucyl tRNA synthetase can ameliorate the pathologic phenotype. See also: HT Hornig-Do et al (February 2014) and E Perli et al (February 2014)