TRMU

Description

The TRMU (tRNA mitochondrial 2-thiouridylase) is a protein-coding gene located on chromosome 22.

Mitochondrial tRNA-specific 2-thiouridylase 1 is an enzyme that in humans is encoded by the TRMU gene. This gene is a member of the trmU family. It encodes a mitochondria-specific tRNA-modifying enzyme that is required for the 2-thio modification of 5-taurinomethyl-2-thiouridine tRNA-Lys on the wobble position of the anticodon.

This enzyme catalyzes the addition of a sulfur atom to uridine at the wobble position (U34) of mitochondrial tRNA(Lys), tRNA(Glu) and tRNA(Gln). This modification is essential for the formation of 5-taurinomethyl-2-thiouridine (tm5s2U) at the wobble position of these tRNAs. ATP is required for this process, specifically to activate the C2 atom of the wobble base.

TRMU is also known as LCAL3, MTO2, MTU1, TRMT, TRMT1.

Associated Diseases

• Deafness, aminoglycoside-induced
• Liver failure, infantile, transient
• Mitochondrial myopathy with reversible cytochrome C oxidase deficiency