MTO1

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Description

The MTO1 (mitochondrial tRNA translation optimization 1) is a protein-coding gene located on chromosome 6.

The MTO1 gene encodes the MTO1 protein, which is a mitochondrial protein found in humans.

The MTO1 protein plays a crucial role in modifying the wobble uridine base within mitochondrial tRNAs. Specifically, it introduces a 5-carboxymethylaminomethyl modification, denoted as mnm(5)s(2)U34.

MTO1 is also known as CGI-02, COXPD10.

Associated Diseases

• Combined oxidative phosphorylation deficiency 10