Ubiquitin recognition factor in ER-associated degradation protein 1 (Ubiquitin fusion degradation protein 1) (UB fusion protein 1)
1_MFSFN 6_ MFDHP 11_ IPRVF 16_ QNRFS 21_ TQYRC 26_ FSVSM 31_ LAGPN 36_ DRSDV 41_ EKGGK 46_ IIMPP 51_ SALDQ 56_ LSRLN 61_ ITYPM 66_ LFKLT 71_ NKNSD 76_ RMTHC 81_ GVLEF 86_ VADEG 91_ ICYLP 96_ HWMMQ 101_ NLLLE 106_ EGGLV 111_ QVESV 116_ NLQVA 121_ TYSKF 126_ QPQSP 131_ DFLDI 136_ TNPKA 141_ VLENA 146_ LRNFA 151_ CLTTG 156_ DVIAI 161_ NYNEK 166_ IYELR 171_ VMETK 176_ PDKAV 181_ SIIEC 186_ DMNVD 191_ FDAPL 196_ GYKEP 201_ ERQVQ 206_ HEEST 211_ EGEAD 216_ HSGYA 221_ GELGF 226_ RAFSG 231_ SGNRL 236_ DGKKK 241_ GVEPS 246_ PSPIK 251_ PGDIK 256_ RGIPN 261_ YEFKL 266_ GKITF 271_ IRNSR 276_ PLVKK 281_ VEEDE 286_ AGGRF 291_ VAFSG 296_ EGQSL 301_RKKGR
1: Essential component of the ubiquitin-dependent proteolytic pathway which degrades ubiquitin fusion proteins. The ternary complex containing UFD1, VCP and NPLOC4 binds ubiquitinated proteins and is necessary for the export of misfolded proteins from the ER to the cytoplasm, where they are degraded by the proteasome. The NPLOC4-UFD1-VCP complex regulates spindle disassembly at the end of mitosis and is necessary for the formation of a closed nuclear envelope. It may be involved in the development of some ectoderm-derived structures (By similarity). Acts as a negative regulator of type I interferon production via the complex formed with VCP and NPLOC4, which binds to RIGI and recruits RNF125 to promote ubiquitination and degradation of RIGI (PubMed:26471729)