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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
    gene 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
    153 from dbSNP Build 142
  • Strain Annotations
    18
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 MGP_129S1SvImJ_G0035071
 protein coding gene Chr9:78955226-78991324 (+)
A/J MGP_AJ_G0035052
 protein coding gene Chr9:76229053-76260665 (+)
AKR/J MGP_AKRJ_G0034980
 protein coding gene Chr9:78033296-78061237 (+)
BALB/cJ MGP_BALBcJ_G0035040
 protein coding gene Chr9:75888931-75917345 (+)
C3H/HeJ MGP_C3HHeJ_G0034752
 protein coding gene Chr9:78552502-78581513 (+)
C57BL/6NJ MGP_C57BL6NJ_G0035561
 protein coding gene Chr9:81085934-81122695 (+)
CAROLI/EiJ MGP_CAROLIEiJ_G0032470
 protein coding gene Chr9:75458885-75482121 (+)
CAST/EiJ MGP_CASTEiJ_G0034066
 protein coding gene Chr9:78701557-78864837 (+)
CBA/J MGP_CBAJ_G0034725
 protein coding gene Chr9:83946179-83980251 (+)
DBA/2J MGP_DBA2J_G0034883
 protein coding gene Chr9:75869487-75901334 (+)
FVB/NJ MGP_FVBNJ_G0034826
 protein coding gene Chr9:75100665-75129147 (+)
LP/J MGP_LPJ_G0034964
 protein coding gene Chr9:79183273-79210588 (+)
NOD/ShiLtJ MGP_NODShiLtJ_G0034868
 protein coding gene Chr9:85133652-85168368 (+)
NZO/HlLtJ MGP_NZOHlLtJ_G0035584
 protein coding gene Chr9:78201809-78231610 (+)
PWK/PhJ MGP_PWKPhJ_G0033773
 protein coding gene Chr9:76065438-76090061 (+)
SPRET/EiJ MGP_SPRETEiJ_G0033610
 protein coding gene Chr9:78401652-78432899 (+)
WSB/EiJ MGP_WSBEiJ_G0034186
 protein coding gene Chr9:78148043-78177551 (+)



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
    neXtProt AC: NX_Q9Y2Z2
    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
    17 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
    10
  • GO References
    4
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
non-membrane-bounded 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
    706
  • 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
    Molecular
    Reagents
    less
    • All nucleic 9
      cDNA 9

      Microarray probesets 4
    Other
    Accession IDs
    less
    MGI:1917779
    References
    more
    • Summaries
      All 43
      Developmental Gene Expression 1
      Diseases 1
      Gene Ontology 4
      Phenotypes 17
    • 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:271287 Fakruddin M, et al., Defective Mitochondrial tRNA Taurine Modification Activates Global Proteostress and Leads to Mitochondrial Disease. Cell Rep. 2018 Jan 9;22(2):482-496
    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
    04/16/2024
    MGI 6.23     		The Jackson Laboratory