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Gene: MTO1 (mitochondrial tRNA translation optimization 1) Homo sapiens
Symbol: MTO1
Name: mitochondrial tRNA translation optimization 1
Description: Exhibits RNA binding activity. Predicted to be involved in mitochondrial tRNA wobble uridine modification and tRNA methylation. Predicted to localize to mitochondrion. Implicated in combined oxidative phosphorylation deficiency 10.
Type: protein-coding
RefSeq Status: REVIEWED
Also known as: CGI-02; COXPD10; homolog of yeast Mto1; mitochondrial MTO1-3; mitochondrial translation optimization 1 homolog; protein MTO1 homolog, mitochondrial
Mus musculus (house mouse) : Mto1 (mitochondrial tRNA translation optimization 1)  MGI  Alliance
Rattus norvegicus (Norway rat) : Mto1 (mitochondrial tRNA translation optimization 1)  Alliance
Chinchilla lanigera (long-tailed chinchilla) : Mto1 (mitochondrial tRNA translation optimization 1)
Pan paniscus (bonobo/pygmy chimpanzee) : MTO1 (mitochondrial tRNA translation optimization 1)
Canis lupus familiaris (dog) : MTO1 (mitochondrial tRNA translation optimization 1)
Ictidomys tridecemlineatus (thirteen-lined ground squirrel) : Mto1 (mitochondrial tRNA translation optimization 1)
Sus scrofa (pig) : MTO1 (mitochondrial tRNA translation optimization 1)
Chlorocebus sabaeus (African green monkey) : MTO1 (mitochondrial tRNA translation optimization 1)
Heterocephalus glaber (naked mole-rat) : Mto1 (mitochondrial tRNA translation optimization 1)
more info ...
Allele / Splice: See ClinVar data
Latest Assembly: GRCh38 - Human Genome Assembly GRCh38
Human AssemblyChrPosition (strand)SourceGenome Browsers
GRCh38.p13 Ensembl673,461,578 - 73,509,236 (+)EnsemblGRCh38hg38GRCh38
GRCh38673,461,737 - 73,509,236 (+)NCBIGRCh38GRCh38hg38GRCh38
GRCh37674,171,454 - 74,211,179 (+)NCBIGRCh37GRCh37hg19GRCh37
Build 36674,228,209 - 74,267,896 (+)NCBINCBI36hg18NCBI36
Celera674,565,658 - 74,605,390 (+)NCBI
Cytogenetic Map6q13NCBI
HuRef671,369,741 - 71,410,063 (+)NCBIHuRef
CHM1_1674,338,243 - 74,377,999 (+)NCBICHM1_1
JBrowse: View Region in Genome Browser (JBrowse)

Disease Annotations
Gene-Chemical Interaction Annotations
Gene Ontology Annotations
Phenotype Annotations
References - curated
References - uncurated


Comparative Map Data
Position Markers
miRNA Target Status


RNA-SEQ Expression


Nucleotide Sequences
Protein Sequences
Clinical Variants

Additional Information

External Database Links
Nomenclature History
More on MTO1
Alliance Gene
Ensembl Gene
JBrowse: hg19 hg38
HGNC Report
NCBI Genome Data Viewer

RGD Object Information
RGD ID: 1318828
Created: 2005-01-12
Species: Homo sapiens
Last Modified: 2020-06-18
Status: ACTIVE


RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.