PRPF19
Ubiquitin-protein ligase which is a core component of several complexes mainly involved pre-mRNA splicing and DNA repair. Required for pre-mRNA splicing as component of the spliceosome. 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. Recruited to RNA polymerase II C-terminal domain (CTD) and the pre-mRNA, it may also couple the transcriptional and spliceosomal machineries. The XAB2 complex, which contains PRPF19, is also involved in pre-mRNA splicing, transcription and transcription-coupled repair. Beside its role in pre-mRNA splicing PRPF19, as part of the PRP19-CDC5L complex, plays a role in the DNA damage response/DDR. It is recruited to the sites of DNA damage by the RPA complex where PRPF19 directly ubiquitinates RPA1 and RPA2. 'Lys-63'-linked polyubiquitination of the RPA complex allows the recruitment of the ATR-ATRIP complex and the activation of ATR, a master regulator of the DNA damage response. May also play a role in DNA double-strand break (DSB) repair by recruiting the repair factor SETMAR to altered DNA. As part of the PSO4 complex may also be involved in the DNA interstrand cross-links/ICLs repair process. In addition, may also mediate 'Lys-48'-linked polyubiquitination of substrates and play a role in proteasomal degradation. May play a role in the biogenesis of lipid droplets. May play a role in neural differentiation possibly through its function as part of the spliceosome. Belongs to the WD repeat PRP19 family. Ubiquitous. Weakly expressed in senescent cells of different tissue origins. Highly expressed in tumor cell lines. Note: This description may include information from UniProtKB.
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Protein type: EC 6.3.2.-; EC 6.3.2.19; Ligase; RNA splicing; Spliceosome; Ubiquitin conjugating system; Ubiquitin ligase |
Chromosomal Location of human Ortholog: 11q12.2 |
Cellular Component:
catalytic step 2 spliceosome; cytoplasm; lipid droplet; nuclear speck; nucleoplasm; nucleus; Prp19 complex; site of double-strand break; spindle; spliceosomal complex; U2-type catalytic step 1 spliceosome; U2-type catalytic step 2 spliceosome
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Molecular Function:
identical protein binding; protein binding; ubiquitin protein ligase activity; ubiquitin-protein transferase activity; ubiquitin-ubiquitin ligase activity
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Biological Process:
DNA damage checkpoint signaling; double-strand break repair via nonhomologous end joining; lipid biosynthetic process; mRNA splicing, via spliceosome; positive regulation of mRNA splicing, via spliceosome; proteasomal protein catabolic process; protein K63-linked ubiquitination; protein localization; protein polyubiquitination; spliceosomal complex assembly; spliceosomal tri-snRNP complex assembly
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Reference #:
Q9UMS4
(UniProtKB)
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Alt. Names/Synonyms: hPso4; NMP200; Nuclear matrix protein 200; nuclear matrix protein NMP200 related to splicing factor PRP19; pre-mRNA processing factor 19; Pre-mRNA-processing factor 19; PRP19; PRP19/PSO4 homolog; PRP19/PSO4 pre-mRNA processing factor 19 homolog; PRP19/PSO4 pre-mRNA processing factor 19 homolog (S. cerevisiae); PRPF19; PSO4; psoralen 4; RING-type E3 ubiquitin transferase PRP19; Senescence evasion factor; SNEV; UBOX4
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Gene Symbols: PRPF19
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Molecular weight:
55,181 Da
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Basal Isoelectric point:
6.14
Predict pI for various phosphorylation states
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