PRPF19
Description
The PRPF19 (pre-mRNA processing factor 19) is a protein-coding gene located on chromosome 11.
Pre-mRNA-processing factor 19 is a protein that in humans is encoded by the PRPF19 gene.
PRPF19 is a ubiquitin-protein ligase that plays a central role in both pre-mRNA splicing and DNA repair. It is a core component of several complexes, including the spliceosome and the PRP19-CDC5L complex. In pre-mRNA splicing, PRPF19 is involved in the assembly, remodeling, and activity of the spliceosome. It specifically mediates Lys-63-linked polyubiquitination of the U4 spliceosomal protein PRPF3, which allows its recognition by PRPF8 and stabilizes the U4/U5/U6 tri-snRNP complex. PRPF19 can also couple transcription and splicing by interacting with RNA polymerase II C-terminal domain (CTD) and pre-mRNA. In DNA repair, PRPF19 is recruited to sites of damage by the RPA complex and directly ubiquitinates RPA1 and RPA2, triggering the recruitment of ATR-ATRIP complex and activation of ATR, a key regulator of DNA damage response. It further promotes DNA double-strand break repair by recruiting the repair factor SETMAR. Additionally, PRPF19 might participate in the repair of DNA interstrand cross-links as part of the PSO4 complex. Beyond these roles, PRPF19 may also mediate Lys-48-linked polyubiquitination of substrates and contribute to proteasomal degradation, participate in lipid droplet biogenesis, and play a potential role in neural differentiation.
PRPF19 is also known as NMP200, PRP19, PSO4, SNEV, UBOX4, hPSO4.
