Entity Details

Primary name MTO1_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionQ9Y2Z2
EntryNameMTO1_HUMAN
FullNameProtein MTO1 homolog, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length717
SequenceStatuscomplete
DateCreated2000-05-30
DateModified2021-06-02

Ontological Relatives

GenesMTO1

GO terms

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GOName
GO:0002098 tRNA wobble uridine modification
GO:0003723 RNA binding
GO:0005739 mitochondrion
GO:0030488 tRNA methylation
GO:0050660 flavin adenine dinucleotide binding
GO:0070899 mitochondrial tRNA wobble uridine modification

Subcellular Location

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Subcellular Location
Mitochondrion

Domains

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DomainNameCategoryType
IPR002218 tRNA uridine 5-carboxymethylaminomethyl modification enzyme MnmG-relatedFamilyFamily
IPR004416 tRNA uridine 5-carboxymethylaminomethyl modification enzyme MnmGFamilyFamily
IPR020595 MnmG-related, conserved siteSiteConserved site
IPR026904 tRNA uridine 5-carboxymethylaminomethyl modification enzyme, C-terminalDomainDomain
IPR036188 FAD/NAD(P)-binding domain superfamilyFamilyHomologous superfamily
IPR040131 MnmG, N-terminal domainDomainDomain

Diseases

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Disease IDSourceNameDescription
614702 OMIMCombined oxidative phosphorylation deficiency 10 (COXPD10)An autosomal recessive disorder resulting in variable defects of mitochondrial oxidative respiration. Affected individuals present in infancy with hypertrophic cardiomyopathy and lactic acidosis. The severity is variable, but can be fatal in the most severe cases. The disease is caused by variants affecting the gene represented in this entry.

Interactions

2 interactions

InteractorPartnerSourcesPublicationsLink
MTO1_HUMANSUV91_HUMANBioGRID, MINT23455924 details
MTO1_HUMANKDM1A_HUMANBioGRID, MINT23455924 details