| Gene Symbol | PRPF19 |
|---|---|
| Entrez Gene | 27339 |
| Alt Symbol | NMP200, PRP19, PSO4, SNEV, UBOX4, hPSO4 |
| Species | Human |
| Gene Type | protein-coding |
| Description | pre-mRNA processing factor 19 |
| Other Description | PRP19/PSO4 homolog|PRP19/PSO4 pre-mRNA processing factor 19 homolog|nuclear matrix protein 200|nuclear matrix protein NMP200 related to splicing factor PRP19|pre-mRNA-processing factor 19|psoralen 4|senescence evasion factor |
| Swissprots | Q9UMS4 |
| Accessions | EAW73904 EAW73905 Q9UMS4 AF086469 AJ131186 CAB51857 AL555169 AW673738 BC008719 AAH08719 BC018665 AAH18665 BC018698 AAH18698 DQ893003 ABM83929 DQ896249 ABM87248 NM_014502 NP_055317 |
| Function | Ubiquitin-protein ligase which is a core component of several complexes mainly involved pre-mRNA splicing and DNA repair. Core component of the PRP19C/Prp19 complex/NTC/Nineteen complex which is part of the spliceosome and participates in its assembly, its remodeling and is required for its activity. During assembly of the spliceosome, mediates 'Lys-63'-linked polyubiquitination of the U4 spliceosomal protein PRPF3. Ubiquitination of PRPF3 allows its recognition by the U5 component PRPF8 and stabilizes the U4/U5/U6 tri-snRNP spliceosomal complex (PubMed:20595234). Recruited to RNA polymerase II C-terminal domain (CTD) and the pre-mRNA, it may also couple the transcriptional and spliceosomal machineries (PubMed:21536736). The XAB2 complex, which contains PRPF19, is also involved in pre- mRNA splicing, transcription and transcription-coupled repair (PubMed:17981804). Beside its role in pre-mRNA splicing PRPF19, as part of the PRP19-CDC5L complex, plays a role in the DNA damage response/D |
| Subcellular Location | Nucleus {ECO:0000269|PubMed:11082287, ECO:0000269|PubMed:12429849, ECO:0000269|PubMed:19188445, ECO:0000269|PubMed:20176811}. Nucleus, nucleoplasm {ECO:0000269|PubMed:11082287}. Cytoplasm, cytoskeleton, spindle {ECO:0000269|PubMed:11082287}. Cytoplasm {ECO:0000269|PubMed:11435423}. Lipid droplet {ECO:0000250|UniProtKB:Q99KP6}. Note=Nucleoplasmic in interphase cells. Irregularly distributed in anaphase cells. In prophase cells, uniformly distributed, but not associated with condensing chromosomes. Found in extrachromosomal regions in metaphase cells. Mainly localized to the mitotic spindle apparatus when chromosomes segregate during anaphase. When nuclei reform during late telophase, uniformly distributed in daughter cells and displays no preferred association with decondensing chromatin. Recruited on damaged DNA at sites of double-strand break. {ECO:0000269|PubMed:11082287, ECO:0000269|PubMed:18263876}. |
| Tissue Specificity | Ubiquitous. Weakly expressed in senescent cells of different tissue origins. Highly expressed in tumor cell lines. {ECO:0000269|PubMed:10404385, ECO:0000269|PubMed:11082287, ECO:0000269|PubMed:11435423, ECO:0000269|PubMed:12960389, ECO:0000269|PubMed:16388800}. |
| Top Pathways | Ubiquitin mediated proteolysis, Spliceosome |