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mitochondrial tRNA translation optimization 1

Summary

Gene Symbol

MTO1

Organism

Homo sapiens (human)

External Links

NCBI Gene

25821

KEGG Gene ID

PubChem

25821

Alliance of Genome Resources

HGNC:19261

Annotation

Keyword

Alternative splicing
Cardiomyopathy
Disease variant
FAD
Methylation
Mitochondrion
Primary mitochondrial disease
Proteomics identification
Reference proteome
Transit peptide
tRNA processing

Proteins

Displaying **all 4** entries
UniProt	Protein Name
Q9Y2Z2	Mitochondrial tRNA translation optimization 1 Protein MTO1 homolog, mitochondrial
A0A7P0TAU1
A0A7P0T8Y9
A0A7P0TA27

Annotation information from UniProt.

Gene Ontology (GO)

Displaying entries **1 - 5** of 6 in total

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GO Term	Evidence Code	PMID
tRNA wobble uridine modification	IEA
tRNA processing	IEA
tRNA methylation
mitochondrial tRNA wobble uridine modification	IMP
mitochondrial tRNA wobble uridine modification	IBA

GO Hierarchy

biological process

cellular process [inference]

metabolic process [inference]

biosynthetic process [inference]

macromolecule biosynthetic process [inference]

gene expression [inference]

mitochondrial gene expression [inference]

mitochondrial RNA processing [inference]

mitochondrial tRNA processing [inference]

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

methylation [inference]

macromolecule methylation [inference]

RNA methylation [inference]

tRNA methylation

macromolecule metabolic process [inference]

macromolecule biosynthetic process [inference]

gene expression [inference]

mitochondrial gene expression [inference]

mitochondrial RNA processing [inference]

mitochondrial tRNA processing [inference]

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

macromolecule modification [inference]

RNA modification [inference]

RNA methylation [inference]

tRNA methylation

tRNA modification [inference]

tRNA wobble base modification [inference]

tRNA wobble uridine modification

mitochondrial tRNA wobble uridine modification

tRNA methylation

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

mitochondrial RNA modification [inference]

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

macromolecule methylation [inference]

RNA methylation [inference]

tRNA methylation

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

mitochondrial RNA metabolic process [inference]

mitochondrial tRNA processing [inference]

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

mitochondrial RNA modification [inference]

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

tRNA metabolic process [inference]

tRNA processing

tRNA modification [inference]

tRNA wobble base modification [inference]

tRNA wobble uridine modification

mitochondrial tRNA wobble uridine modification

tRNA methylation

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

mitochondrial tRNA processing [inference]

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

RNA modification [inference]

RNA methylation [inference]

tRNA methylation

tRNA modification [inference]

tRNA wobble base modification [inference]

tRNA wobble uridine modification

mitochondrial tRNA wobble uridine modification

tRNA methylation

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

mitochondrial RNA modification [inference]

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

primary metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

mitochondrial RNA metabolic process [inference]

mitochondrial tRNA processing [inference]

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

mitochondrial RNA modification [inference]

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

tRNA metabolic process [inference]

tRNA processing

tRNA modification [inference]

tRNA wobble base modification [inference]

tRNA wobble uridine modification

mitochondrial tRNA wobble uridine modification

tRNA methylation

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

mitochondrial tRNA processing [inference]

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

RNA modification [inference]

RNA methylation [inference]

tRNA methylation

tRNA modification [inference]

tRNA wobble base modification [inference]

tRNA wobble uridine modification

mitochondrial tRNA wobble uridine modification

tRNA methylation

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

mitochondrial RNA modification [inference]

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

RNA processing [inference]

mitochondrial RNA processing [inference]

mitochondrial tRNA processing [inference]

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

tRNA processing

tRNA modification [inference]

tRNA wobble base modification [inference]

tRNA wobble uridine modification

mitochondrial tRNA wobble uridine modification

tRNA methylation

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

mitochondrial tRNA processing [inference]

mitochondrial tRNA modification [inference]

mitochondrial tRNA wobble uridine modification

Displaying entries **1 - 5** of 9 in total

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GO Term	Evidence Code	PMID
mitochondrion	ISS
mitochondrion	IBA
mitochondrion	IEA
mitochondrion	HTP
cytosol

GO Hierarchy

cellular component

protein-containing complex [inference]

catalytic complex [inference]

transferase complex

GTPBP3-MTO1 complex

cellular anatomical structure [inference]

cytosol

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

Displaying **all 4** entries
GO Term	Evidence Code	PMID
RNA binding	HDA
flavin adenine dinucleotide binding	IEA
tRNA 5-taurinomethyluridine synthase activity	IMP
tRNA 5-taurinomethyluridine synthase activity	IBA

GO Hierarchy

molecular function

catalytic activity [inference]

transferase activity [inference]

tRNA 5-taurinomethyluridine synthase activity

catalytic activity, acting on a nucleic acid [inference]

catalytic activity, acting on RNA [inference]

catalytic activity, acting on a tRNA [inference]

tRNA 5-taurinomethyluridine synthase activity

binding [inference]

nucleic acid binding [inference]

RNA binding

small molecule binding [inference]

ion binding [inference]

anion binding [inference]

flavin adenine dinucleotide binding

heterocyclic compound binding [inference]

nucleotide binding [inference]

flavin adenine dinucleotide binding

nucleoside phosphate binding [inference]

nucleotide binding [inference]

flavin adenine dinucleotide binding

Gene Ontology information from NCBI Gene and UniProt.

Disease

Disease Ontology

Displaying 1 entry
DO ID	Disease Name	Source
DOID:0111480	combined oxidative phosphorylation deficiency 10	Alliance of Genome Resources

Ortholog

Displaying 1 entry
Species	Gene ID	Alliance of Genome Resources
Mus musculus	68291	MGI:1915541

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Last updated: April 6, 2026

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