TRMU (O75648) variants and mutations

TRMU (also known as O75648) is a human protein-coding gene encoding a mitochondrial tRNA-specific 2-thiouridylase 1 protein. A mitochondrial tRNA-modifying enzyme that adds sulfur to wobble-position uridines in several mitochondrial tRNAs. This modification supports accurate mitochondrial protein synthesis, and TRMU variants are associated with aminoglycoside-related deafness and transient infantile liver failure. This analysis covers 801 TRMU variants and mutations. Of these, 98% have computational variant effect predictions. Disease context includes acute infantile liver failure due to synthesis defect of mtDNA-encoded proteins, Mitochondrial non-syndromic sensorineural deafness with susceptibility to aminog, and deafness, aminoglycoside-induced. Example TRMU variants include M1?, M1K, and M1R.

Variant analysis overview

Variant and mutation evidence

Clinical, disease, and population context

Protein structure and variant hotspots

Data sources

Evidence in this analysis draws on EBI Proteins Variation, UniProt, gnomAD v4, EuropePMC, 3D Hotspot Analysis, Interaction Network Analysis, AlphaFold DB, gnomAD constraint, Open Targets, ClinGen, PharmGKB, MaveDB, LitVar.

Notable TRMU variants

Examples include M1?, M1K, M1R, M1T, Q2*, Q2E, Q2H, Q2R. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.