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Proteins/UNIPROT:Q96CM3
Catalyzes uridine to pseudouridine isomerization (pseudouridylation) of different mitochondrial RNA substrates. Acts on position 1397 in 16S mitochondrial ribosomal RNA (16S mt-rRNA). This modification is required for the assembly of 16S mt-rRNA into a functional mitochondrial ribosome. As a component of a functional protein-RNA module, consisting of RCC1L, NGRN, RPUSD3, RPUSD4, TRUB2, FASTKD2 and 16S mt-rRNA, controls 16S mt-rRNA abundance and is required for intra-mitochondrial translation. Acts on position 39 in mitochondrial tRNA(Phe). Also catalyzes pseudouridylation of mRNAs in nucleus: acts as a regulator of pre-mRNA splicing by mediating pseudouridylation of pre-mRNAs at locations associated with alternatively spliced regions. Pseudouridylation of pre-mRNAs near splice sites directly regulates mRNA splicing and mRNA 3'-end processing..
Function from UniProtKB (Q96CM3), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
9 statements
9 statements · 4 reference databases · Subject: RPUSD4 · Sources: gene ontology, Hetionet, Human-GEM, Reactome
4 reactions
water+MAM02807cCHEBI:17797+uracil
Catalysed by RPUSD4
Reaction pageReversibleBalancedEC 4.2.1.70cytosol
Human-GEM MAR07744CC BY 4.0Reference data
CHEBI:65315CHEBI:65314
Reaction pageDirection not assertedBalanced
Rhea RHEA:47036CC BY 4.0Reference data
a uridine in tRNA
1 name
Resolves to: RPUSD4
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:54572CC BY 4.0Reference data
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