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Article

The 2-thiouridylase function of the human MTU1 (TRMU) enzyme is dispensable for mitochondrial translation.

Human molecular genetics - 1 Dec 2011

Sasarman Florin, Antonicka Hana, Horvath Rita, Shoubridge Eric A

Abstract excerpt

MTU1 (TRMU) is a mitochondrial enzyme responsible for the 2-thiolation of the wobble U in tRNA(Lys), tRNA(Glu) and tRNA(Gln), a post-transcriptional modification believed to be important for accurate and efficient synthesis of the 13 respiratory chain subunits encoded by mtDNA. Mutations in MTU1 are associated with acute infantile liver failure, and this has been ascribed to a transient lack of cysteine, the...

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PMID: 21890497
DOI: 10.1093/hmg/ddr397

Genes

TRMU

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

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The 2-thiouridylase function of the human MTU1 (TRMU) enzyme is dispensable for mitochondrial translation.PMID 21890497Read ↗

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TRMT2B is responsible for both tRNA and rRNA m <sup>5</sup> U-methylation in human mitochondriaSemantic proximity Read ↗

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