Recombinant Human Pre-mRNA-processing factor 19 (PRPF19)

Uniprot Id

Q9UMS4

Target Species

Human

Target Name

PRPF19

Target Full Name

Pre-mRNA-processing factor 19

Target Function

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.

Target Subcellular Location

Nucleus. Nucleus, nucleoplasm. Cytoplasm, cytoskeleton, spindle. Cytoplasm. Lipid droplet.

Target Protein Families

WD repeat PRP19 family

Target Tissue Specificity

Ubiquitous. Weakly expressed in senescent cells of different tissue origins. Highly expressed in tumor cell lines.

Target 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 (S. cerevisiae); PRP19_HUMAN; PRPF19; PSO4; psoralen 4; Senescence evasion factor; SNEV; UBOX4

Target Background

Enables identical protein binding activity and ubiquitin-ubiquitin ligase activity. Involved in several processes, including DNA damage checkpoint signaling; cellular protein metabolic process; and mRNA splicing, via spliceosome. Acts upstream of or within protein polyubiquitination. Located in cytoplasm; nuclear speck; and site of double-strand break. Part of Prp19 complex and U2-type catalytic step 2 spliceosome. Colocalizes with DNA replication factor A complex.