Proteins/UNIPROT:Q9Y2Z2

MTO1

ProteinUNIPROT:Q9Y2Z25-taurinomethyluridine-[tRNA] synthase subunit MTO1, mitochondrial

Component of the GTPBP3-MTO1 complex that catalyzes the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position (U34) of mitochondrial tRNAs (mt-tRNAs), which plays a role in mt-tRNA decoding and mitochondrial translation. Taum(5)U formation on mammalian mt-tRNA requires the presence of both GTPBP3-mediated GTPase activity and MTO1 catalytic activity..

Function from UniProtKB (Q9Y2Z2), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

2 rows

mitochondrial disease has a germline basis in MTO1DiseaseGenCC HGNC:19261|MONDO:0044970Inferred only
mitochondrial hypertrophic cardiomyopathy with lactic acidosis due to MTO1 deficiency has a germline basis in MTO1DiseaseGenCC HGNC:19261|MONDO:0013865Inferred only

Statements

14 statements

14 statements · 4 reference databases · Subject: MTO1 · Sources: Hetionet, Reactome, Rhea, UniProt

Order

MTO1 participates in Metabolism of RNAPathwayReactome R-HSA-8953854Inferred only
MTO1 participates in tRNA modification in the mitochondrionPathwayReactome R-HSA-6787450Inferred only
MTO1 participates in tRNA processingPathwayReactome R-HSA-72306Inferred only
ATG12 increases the expression of MTO1ProteinHetionet hetionet:regulates:9140-25821Inferred only
BECN1 increases the expression of MTO1ProteinHetionet hetionet:regulates:8678-25821Inferred only
CCNF increases the expression of MTO1ProteinHetionet hetionet:regulates:899-25821Inferred only
ROS1 increases the expression of MTO1ProteinHetionet hetionet:regulates:6098-25821Inferred only
SLC35A1 decreases the expression of MTO1ProteinHetionet hetionet:regulates:10559-25821Inferred only
UQCRC1 increases the expression of MTO1ProteinHetionet hetionet:regulates:7384-25821Inferred only
VAT1 decreases the expression of MTO1ProteinHetionet hetionet:regulates:10493-25821Inferred only
MTO1 requires the cofactor FAD(3-)CompoundUniProt Q9Y2Z2Inferred only
5,10-methylenetetrahydrofolate is a substrate of MTO1CompoundRhea RHEA:83279Inferred only

Show the remaining 2

A is a substrate of MTO1CompoundRhea RHEA:83279Inferred only
taurine is a substrate of MTO1CompoundRhea RHEA:83279Inferred only

Reactions

1 reaction

CHEBI:65315+5,10-methylenetetrahydrofolate+taurine+GTP+A+waterCHEBI:87172+7,8-dihydrofolate+GDP+AH2+hydrogenphosphate+hydron
Catalysed by MTO1
Reaction pageLeft to rightBalanced
Rhea RHEA:83279CC BY 4.0Reference data

Other names

3 names

Resolves to: MTO1

Also called: Mitochondrial tRNA translation optimization 1MTO1 homolog, mitochondrialProtein MTO1 homolog, mitochondrial

← Every protein Find it in the graph →