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Mto1 Gene Detail
Summary
  • Symbol
    Mto1
  • Name
    mitochondrial tRNA translation optimization 1
  • Synonyms
    2310039H01Rik, 5730419A02Rik
  • Feature Type
    protein coding gene
  • IDs
    MGI:1915541
    NCBI Gene: 68291
  • Alliance
    genome feature page
  • Transcription Start Sites
    1 TSS
Location &
Maps
more
  • Sequence Map
    Chr9:78355372-78381447 bp, + strand
    From NCBI annotation of GRCm39
  • View this region in JBrowse
  • Genome Browsers
    Ensembl | UCSC | NCBI
  • Genetic Map
    Chromosome 9, 43.65 cM, cytoband E1
  • Mapping Data
    3 experiments
Strain
Comparison
more
  • SNPs within 2kb
    696 from dbSNP Build 142
  • Strain Annotations
    19
For selected strains:
Strain Gene Model ID Feature Type Coordinates Select Strains
C57BL/6J MGI_C57BL6J_1915541
 protein coding gene Chr9:78355372-78382630 (+)
129S1/SvImJ ENSMUSG00200018456
 protein coding gene Chr9:75506359-75532247 (+)
A/J ENSMUSG00195009792
 protein coding gene Chr9:75290524-75316477 (+)
AKR/J ENSMUSG00220014876
 protein coding gene Chr9:75396835-75422784 (+)
BALB/cJ ENSMUSG00180016909
 protein coding gene Chr9:75404062-75430014 (+)
C3H/HeJ ENSMUSG00175011502
 protein coding gene Chr9:75462917-75488868 (+)
C57BL/6NJ ENSMUSG00215009313
 protein coding gene Chr9:75342275-75368220 (+)
CAROLI/EiJ MGP_CAROLIEiJ_G0032470
 protein coding gene Chr9:75458885-75482121 (+)
CAST/EiJ ENSTCUG00005008598
 protein coding gene Chr9:75041877-75068075 (+)
CBA/J ENSMUSG00210013223
 protein coding gene Chr9:75495839-75521787 (+)
DBA/2J ENSMUSG00185006500
 protein coding gene Chr9:75524153-75550103 (+)
FVB/NJ ENSMUSG00205019619
 protein coding gene Chr9:75684864-75710817 (+)
JF1/MsJ ENSUMUG00000034992
 protein coding gene Chr9:75432480-75458345 (+)
LP/J ENSMUSG00230024315
 protein coding gene Chr9:75657952-75683838 (+)
NOD/ShiLtJ ENSMUSG00190025063
 protein coding gene Chr9:75600238-75626188 (+)
NZO/HlLtJ ENSMUSG00225024768
 protein coding gene Chr9:75822102-75848052 (+)
PWK/PhJ ENSLUMG00010015636
 protein coding gene Chr9:75365536-75391519 (+)
SPRET/EiJ ENSMSPG00010003942
 protein coding gene Chr9:76592817-76618361 (+)
WSB/EiJ ENSIUOG00005023579
 protein coding gene Chr9:74929444-74955397 (+)



Homology
more
  • Human Ortholog
    MTO1, mitochondrial tRNA translation optimization 1
  • Vertebrate Orthologs
    3
Vertebrate Orthology Source
Alliance of Genome Resources
  • Human Ortholog
    MTO1, mitochondrial tRNA translation optimization 1
  • Synonyms
    CGI-02, COXPD10
  • Links
    NCBI Gene ID: 25821
    UniProt: Q9Y2Z2

  • Chr Location
    6q13; chr6:73461578-73509236 (+)  GRCh38
Human Diseases
more
  • Diseases
    1 with Mto1 mouse models; 1 with human MTO1 associations

Human Disease Mouse Models
      
IDs
 View 1 model
      
IDs
Click on a disease name to see all genes associated with that disease.

  • Mutations/Alleles
    1 with disease annotations
  • References
    1 with disease annotations
Mutations,
Alleles, and
Phenotypes
less
  • Phenotype Summary
    28 phenotypes from 1 allele in 1 genetic background
    19 phenotype references
Phenotype Overview

adipose tissue
behavior/neurological
cardiovascular system
cellular
craniofacial
digestive/alimentary system
embryo
endocrine/exocrine glands
growth/size/body
hearing/vestibular/ear
hematopoietic system
homeostasis/metabolism
integument
immune system
limbs/digits/tail
liver/biliary system
mortality/aging
muscle
nervous system
pigmentation
renal/urinary system
reproductive system
respiratory system
skeleton
taste/olfaction
neoplasm
vision/eye

Click cells to view annotations.
Homozygotes for a hypomorphic allele show bradycardia, cardiomyopathy, worsening of arrhythmias during induction and reversal of anesthesia, and mitochondrial abnormalities.
Gene Ontology
(GO)
Classifications
less
  • All GO Annotations
    23
  • GO References
    9
Molecular Function

carbohydrate derivative binding
cytoskeletal protein binding
DNA binding
enzyme regulator
hydrolase
ligase
lipid binding
oxidoreductase
RNA binding
signaling receptor activity
signaling receptor binding
transcription
transferase
transporter
Biological Process

carbohydrate derivative metabolism
cell differentiation
cell population proliferation
cellular component organization
DNA-templated transcription
establishment of localization
homeostatic process
immune system process
lipid metabolic process
programmed cell death
protein metabolic process
response to stimulus
signaling
system development
Cellular Component

cell projection
cytoplasmic vesicle
cytoskeleton
cytosol
endoplasmic reticulum
endosome
extracellular region
Golgi apparatus
mitochondrion
membraneless organelle
nucleus
organelle envelope
organelle lumen
plasma membrane
protein-containing complex
synapse
vacuole
Click cells to view annotations.
Expression
less
Expression Overview

early conceptus
embryo ectoderm
embryo endoderm
embryo mesoderm
embryo mesenchyme
extraembryonic component
alimentary system
auditory system
branchial arches
cardiovascular system
connective tissue
endocrine system
exocrine system
hemolymphoid system
integumental system
limbs
liver and biliary system
musculoskeletal system
nervous system
olfactory system
reproductive system
respiratory system
urinary system
visual system
Click cells to view annotations.


  • Assay Results
    597
  • cDNA Data
    9
  • Literature Summary
    1
  • Comparison Matrix
    Gene Expression + Phenotype
    • Sequences &
    Gene Models
    less
    Representative SequencesLengthStrain/SpeciesFlank
    genomic 68291 NCBI Gene Model | MGI Sequence Detail 26076 C57BL/6J ±  kb
    transcript NM_026658 RefSeq | MGI Sequence Detail 2364 C57BL/6  
    polypeptide Q923Z3 UniProt | EBI | MGI Sequence Detail 669 Not Applicable  
    For the selected sequence
    Protein
    Information
    less
    • UniProt
      4 Sequences
    • Protein Ontology
      PR:000010743 protein MTO1, mitochondrial
      (term hierarchy)
    • InterPro Domains
      IPR036188 FAD/NAD(P)-binding domain superfamily
      IPR040131 MnmG, N-terminal domain
      IPR020595 MnmG-related, conserved site
      IPR049312 tRNA uridine 5-carboxymethylaminomethyl modification enzyme, C-terminal, N-terninal subdomain
      IPR047001 tRNA uridine 5-carboxymethylaminomethyl modification enzyme, C-terminal subdomain
      IPR044920 tRNA uridine 5-carboxymethylaminomethyl modification enzyme, C-terminal subdomain superfamily
      IPR004416 tRNA uridine 5-carboxymethylaminomethyl modification enzyme MnmG
      IPR026904 tRNA uridine 5-carboxymethylaminomethyl modification enzyme MnmG, C-terminal
      IPR002218 tRNA uridine 5-carboxymethylaminomethyl modification enzyme MnmG-related
    • GlyGen
      Q923Z3 1 site, 1 O-linked glycan (1 site)
    Molecular
    Reagents
    less
    • All nucleic 9
      cDNA 9

      Microarray probesets 4
    Other
    Accession IDs
    less
    MGI:1917779
    References
    more
    • Summaries
      All 51
      Developmental Gene Expression 1
      Diseases 1
      Gene Ontology 9
      Phenotypes 19
    • Earliest
      J:12013 Russell LB, Definition of functional units in a small chromosomal segment of the mouse and its use in interpreting the nature of radiation-induced mutations. Mutat Res. 1971 Jan;11(1):107-23
    • Latest
      J:361940 Morishima T, et al., Mitochondrial translation regulates terminal erythroid differentiation by maintaining iron homeostasis. Sci Adv. 2025 Feb 21;11(8):eadu3011
    Contributing Projects:
    Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
    Citing These Resources
    Funding Information
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    Send questions and comments to User Support.     		last database update
    09/01/2026
    MGI 6.24     		The Jackson Laboratory