Function | 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. |
Protein Name | Pseudouridylate Synthase Rpusd4 - MitochondrialRna Pseudouridylate Synthase Domain-Containing Protein 4 |
Cellular Localisation | Mitochondrion MatrixNucleusCytoplasmMainly Localizes To MitochondrionLocalizes To Mitochondrial Rna GranulesPlatforms For Post-Transcriptional Rna Modification And Ribosome AssemblyAlso Found In Nucleus And Cytoplasm |
Alternative Antibody Names | Anti-Pseudouridylate Synthase Rpusd4 - Mitochondrial antibodyAnti-Rna Pseudouridylate Synthase Domain-Containing Protein 4 antibodyAnti-RPUSD4 antibody |
Information sourced from Uniprot.org