Proteins/UNIPROT:Q6P087

RPUSD3

ProteinUNIPROT:Q6P087

Catalyzes uridine to pseudouridine isomerization (pseudouridylation) of specific mitochondrial mRNAs (mt-mRNAs), a post-transcriptional modification necessary for their translation. Acts at position 390 in COXI mt-mRNA and at position 697-699 in mitochondrial COXIII mt-mRNA. As a component of a functional protein-RNA module, consisting of RCC1L, NGRN, RPUSD3, RPUSD4, TRUB2, FASTKD2 and 16S mitochondrial ribosomal RNA (16S mt-rRNA), controls 16S mt-rRNA abundance and may play a role in mitochondrial ribosome biogenesis..

Function from UniProtKB (Q6P087), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

Statements

9 statements

9 statements · 3 reference databases · Subject: RPUSD3 · Sources: gene ontology, Hetionet, Reactome

Order

RPUSD3 covaries with TRIOBPProteinHetionet hetionet:covaries:11078-285367Inferred only
MRPL58 interacts with RPUSD3ProteinHetionet hetionet:interacts:285367-3396Inferred only
UBC interacts with RPUSD3ProteinHetionet hetionet:interacts:285367-7316Inferred only
RPUSD3 participates in Metabolism of RNAPathwayReactome R-HSA-8953854Inferred only
RPUSD3 participates in Mitochondrial mRNA modificationPathwayReactome R-HSA-9937008Inferred only
RPUSD3 participates in rRNA modification in the mitochondrionPathwayReactome R-HSA-6793080Inferred only
RPUSD3 participates in rRNA processingPathwayReactome R-HSA-72312Inferred only
RPUSD3 participates in rRNA processing in the mitochondrionPathwayReactome R-HSA-8868766Inferred only
RPUSD3 regulates positive regulation of mitochondrial translationBiological processgene ontology Q6P087|GO:0070131Inferred only

Reactions

1 reaction

a uridine in mRNAa pseudouridine in mRNA
Catalysed by TRUB2 or RPUSD3 or RPUSD2 or TRUB1 or RPUSD4 or PUS7 or PUS7L or PUS1
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:56644CC BY 4.0Reference data

Other names

1 name

Resolves to: RPUSD3

Also called: RNA pseudouridylate synthase domain-containing protein 3

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