Pre-mRNA-processing factor 19 (EC 2.3.2.27) (Nuclear matrix protein 200) (PRP19/PSO4 homolog) (hPso4) (RING-type E3 ubiquitin transferase PRP19) (Senescence evasion factor)
1_MSLIC 6_ SISNE 11_ VPEHP 16_ CVSPV 21_ SNHVY 26_ ERRLI 31_ EKYIA 36_ ENGTD 41_ PINNQ 46_ PLSEE 51_ QLIDI 56_ KVAHP 61_ IRPKP 66_ PSATS 71_ IPAIL 76_ KALQD 81_ EWDAV 86_ MLHSF 91_ TLRQQ 96_ LQTTR 101_ QELSH 106_ ALYQH 111_ DAACR 116_ VIARL 121_ TKEVT 126_ AAREA 131_ LATLK 136_ PQAGL 141_ IVPQA 146_ VPSSQ 151_ PSVVG 156_ AGEPM 161_ DLGEL 166_ VGMTP 171_ EIIQK 176_ LQDKA 181_ TVLTT 186_ ERKKR 191_ GKTVP 196_ EELVK 201_ PEELS 206_ KYRQV 211_ ASHVG 216_ LHSAS 221_ IPGIL 226_ ALDLC 231_ PSDTN 236_ KILTG 241_ GADKN 246_ VVVFD 251_ KSSEQ 256_ ILATL 261_ KGHTK 266_ KVTSV 271_ VFHPS 276_ QDLVF 281_ SASPD 286_ ATIRI 291_ WSVPN 296_ ASCVQ 301_ VVRAH 306_ ESAVT 311_ GLSLH 316_ ATGDY 321_ LLSSS 326_ DDQYW 331_ AFSDI 336_ QTGRV 341_ LTKVT 346_ DETSG 351_ CSLTC 356_ AQFHP 361_ DGLIF 366_ GTGTM 371_ DSQIK 376_ IWDLK 381_ ERTNV 386_ ANFPG 391_ HSGPI 396_ TSIAF 401_ SENGY 406_ YLATA 411_ ADDSS 416_ VKLWD 421_ LRKLK 426_ NFKTL 431_ QLDNN 436_ FEVKS 441_ LIFDQ 446_ SGTYL 451_ ALGGT 456_ DVQIY 461_ ICKQW 466_ TEILH 471_ FTEHS 476_ GLTTG 481_ VAFGH 486_ HAKFI 491_ ASTGM 496_DRSLK
1: 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 (PubMed:28076346, PubMed:28502770, PubMed:29301961, PubMed:29360106, PubMed:30705154). 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/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 (PubMed:24332808). May also play a role in DNA double-strand break (DSB) repair by recruiting the repair factor SETMAR to altered DNA (PubMed:18263876). As part of the PSO4 complex may also be involved in the DNA interstrand cross-links/ICLs repair process (PubMed:16223718). In addition, may also mediate 'Lys-48'-linked polyubiquitination of substrates and play a role in proteasomal degradation (PubMed:11435423). May play a role in the biogenesis of lipid droplets (By similarity). May play a role in neural differentiation possibly through its function as part of the spliceosome (By similarity)