RPUSD3
Function
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 (PubMed:27974379). 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 (PubMed:27667664).
Sequence Similarities
Belongs to the pseudouridine synthase RluA family.
Cellular localization
- Mitochondrion matrix
- Localizes to mitochondrial RNA granules, platforms for post-transcriptional RNA modification and ribosome assembly.
Alternative names
Mitochondrial mRNA pseudouridine synthase RPUSD3, RNA pseudouridylate synthase domain-containing protein 3, RPUSD3