RPUSD4
Function
Catalyzes uridine to pseudouridine isomerization (pseudouridylation) of different mitochondrial RNA substrates (PubMed:27974379, PubMed:28082677). Acts on position 1397 in 16S mitochondrial ribosomal RNA (16S mt-rRNA) (PubMed:27974379). This modification is required for the assembly of 16S mt-rRNA into a functional mitochondrial ribosome (PubMed:27974379). 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 (PubMed:27667664). Acts on position 39 in mitochondrial tRNA(Phe) (PubMed:28082677). 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 (PubMed:35051350). Pseudouridylation of pre-mRNAs near splice sites directly regulates mRNA splicing and mRNA 3'-end processing (PubMed:35051350).
Sequence Similarities
Belongs to the pseudouridine synthase RluA family.
Cellular localization
- Mitochondrion matrix
- Nucleus
- Cytoplasm
- Mainly localizes to mitochondrion (PubMed:23435261, PubMed:27974379, PubMed:28082677). Localizes to mitochondrial RNA granules, platforms for post-transcriptional RNA modification and ribosome assembly (PubMed:27974379, PubMed:28082677). Also found in nucleus and cytoplasm (PubMed:23435261).
Alternative names
RNA pseudouridylate synthase domain-containing protein 4, RPUSD4