This gene encodes a mitochondrial protein thought to be involved in mitochondrial tRNA modification. The encoded protein may also play a role in the expression of the non-syndromic and aminoglycoside-induced deafness phenotypes associated with a specific mutation in the mitochondrial 12S rRNA gene. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq].
CGI-02; COXPD10; homolog of yeast Mto1; mitochondrial MTO1-3; mitochondrial translation optimization 1 homolog; mitochondrial translation optimization 1 homolog S. cerevisiae; Protein MTO1 homolog, mitochondrial