Mto1 mitochondrial tRNA translation optimization 1 [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Mto1provided by MGI
Official Full Name: mitochondrial tRNA translation optimization 1provided by MGI
Primary source: MGI:MGI:1915541
See related: Ensembl:ENSMUSG00000032342 AllianceGenome:MGI:1915541
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: 2310039H01Rik; 5730419A02Rik
Summary: 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). [provided by Alliance of Genome Resources, Nov 2024]
Expression: Ubiquitous expression in adrenal adult (RPKM 18.7), ovary adult (RPKM 7.4) and 28 other tissues See more
Orthologs: human all
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Genomic context

Location:: 9 E1; 9 43.65 cM

Exon count:: 12

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	9	NC_000075.7 (78355372..78381447)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	9	NC_000075.6 (78443479..78474165)

Chromosome 9 - NC_000075.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: Mouse ENCODE transcriptome data Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

MTO1 mediates tRNA modification and controls mitochondrial translation rate in a highly tissue-specific manner associated with tissue-specific oxidative phosphorylation defects.
Title: MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention.
here described is an MTO1-deficient mouse model generated by gene trap mutagenesis that mirrors the human phenotype showing complex I defect and cardiomyopathy.
Title: MTO1-deficient mouse model mirrors the human phenotype showing complex I defect and cardiomyopathy.
These observations suggest that the mouse MTO1 is a structural and functional homolog of yeast MTO1, thereby playing a role in the mitochondrial tRNA modification and protein synthesis.
Title: Identification and characterization of mouse MTO1 gene related to mitochondrial tRNA modification.

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Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Gene trapped (2)
Targeted (3) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

AA987151 (e-PCR)
- UniSTS:160608

D10Mit157.2 (e-PCR), detects polymorphism
- UniSTS:126061

RH143356 (e-PCR)
- UniSTS:232603

NoName (e-PCR)
- UniSTS:236205

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables flavin adenine dinucleotide binding	IBA Inferred from Biological aspect of Ancestor more info
enables flavin adenine dinucleotide binding	IEA Inferred from Electronic Annotation more info

Process	Evidence Code	Pubs
involved_in mitochondrial tRNA wobble uridine modification	IBA Inferred from Biological aspect of Ancestor more info
acts_upstream_of_or_within mitochondrial tRNA wobble uridine modification	IGI Inferred from Genetic Interaction more info	PubMed
involved_in tRNA methylation	IBA Inferred from Biological aspect of Ancestor more info
acts_upstream_of_or_within tRNA wobble uridine modification	IGI Inferred from Genetic Interaction more info	PubMed

Component	Evidence Code	Pubs
is_active_in cytosol	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrion	HDA more info	PubMed
is_active_in mitochondrion	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrion	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrion	ISO Inferred from Sequence Orthology more info	PubMed

General protein information

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Preferred Names: protein MTO1 homolog, mitochondrial

Names: mitochondrial translation optimization 1 homolog

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_026658.2 → NP_080934.2 protein MTO1 homolog, mitochondrial precursor

See identical proteins and their annotated locations for NP_080934.2

Status: VALIDATED

Source sequence(s)

AF369902, BB811598, BY735135, CF173757

Consensus CDS

CCDS23363.1

UniProtKB/Swiss-Prot

Q923Z3, Q9D2Q5

UniProtKB/TrEMBL

G5E889

Related

ENSMUSP00000034896.7, ENSMUST00000034896.13

Conserved Domains (3) summary

PRK05192
Location:56 → 669

PRK05192; tRNA uridine 5-carboxymethylaminomethyl modification enzyme GidA; Validated

pfam13932
Location:437 → 653

GIDA_assoc; GidA associated domain

cl21454
Location:357 → 455

NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins