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Protein Coding Gene : Mto1 mitochondrial tRNA translation optimization 1

Primary Identifier  MGI:1915541 Organism  mouse, laboratory
Chromosome  9 NCBI Gene Number  68291
Mgi Type  protein coding gene
description  FUNCTION: Automated description from the Alliance of Genome Resources (Release 7.1.0)

Predicted to enable flavin adenine dinucleotide binding activity. Acts upstream of or within mitochondrial tRNA wobble uridine modification. Located in mitochondrion. Used to study combined oxidative phosphorylation deficiency. Human ortholog(s) of this gene implicated in combined oxidative phosphorylation deficiency 10. Orthologous to human MTO1 (mitochondrial tRNA translation optimization 1).
PHENOTYPE: Homozygotes for a hypomorphic allele show bradycardia, cardiomyopathy, worsening of arrhythmias during induction and reversal of anesthesia, and mitochondrial abnormalities. [provided by MGI curators]
  • synonyms:
  • Mto1,
  • mitochondrial tRNA translation optimization 1,
  • 2310039H01Rik,
  • 5730419A02Rik,
  • RIKEN cDNA 5730419A02 gene,
  • MGI:1917779,
  • RIKEN cDNA 2310039H01 gene

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2 Involved In Mutations

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Canonical gene --> Transcripts in specific strains.

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