Ubiquilin-4 (Ataxin-1 interacting ubiquitin-like protein) (A1Up) (Ataxin-1 ubiquitin-like-interacting protein A1U) (Connexin43-interacting protein of 75 kDa) (CIP75)
1_MAEPS 6_ GAETR 11_ PPIRV 16_ TVKTP 21_ KDKEE 26_ IVICD 31_ RASVK 36_ EFKEE 41_ ISRRF 46_ KAQQD 51_ QLVLI 56_ FAGKI 61_ LKDGD 66_ TLNQH 71_ GIKDG 76_ LTVHL 81_ VIKTP 86_ QKAQD 91_ PAAAT 96_ ASSPS 101_ TPDPA 106_ SAPST 111_ TPASP 116_ ATPAQ 121_ PSTSG 126_ SASSD 131_ AGSGS 136_ RRSSG 141_ GGPSP 146_ GAGEG 151_ SPSAT 156_ ASILS 161_ GFGGI 166_ LGLGS 171_ LGLGS 176_ ANFME 181_ LQQQM 186_ QRQLM 191_ SNPEM 196_ LSQIM 201_ ENPLV 206_ QDMMS 211_ NPDLM 216_ RHMIM 221_ ANPQM 226_ QQLME 231_ RNPEI 236_ SHMLN 241_ NPELM 246_ RQTME 251_ LARNP 256_ AMMQE 261_ MMRNQ 266_ DRALS 271_ NLESI 276_ PGGYN 281_ ALRRM 286_ YTDIQ 291_ EPMFS 296_ AAREQ 301_ FGNNP 306_ FSSLA 311_ GNSDS 316_ SSSQP 321_ LRTEN 326_ REPLP 331_ NPWSP 336_ SPPTS 341_ QAPGS 346_ GGEGT 351_ GGSGT 356_ SQVHP 361_ TVSNP 366_ FGINA 371_ ASLGS 376_ GMFNS 381_ PEMQA 386_ LLQQI 391_ SENPQ 396_ LMQNV 401_ ISAPY 406_ MRSMM 411_ QTLAQ 416_ NPDFA 421_ AQMMV 426_ NVPLF 431_ AGNPQ 436_ LQEQL 441_ RLQLP 446_ VFLQQ 451_ MQNPE 456_ SLSIL 461_ TNPRA 466_ MQALL 471_ QIQQG 476_ LQTLQ 481_ TEAPG 486_ LVPSL 491_ GSFGI 496_ SRTPA 501_ PSAGS 506_ NAGST 511_ PEAPT 516_ SSPAT 521_ PATSS 526_ PTGAS 531_ SAQQQ 536_ LMQQM 541_ IQLLA 546_ GSGNS 551_ QVQTP 556_ EVRFQ 561_ QQLEQ 566_ LNSMG 571_ FINRE 576_ ANLQA 581_ LIATG 586_ GDINA 591_ AIERL 596_LGSQL
1: Regulator of protein degradation that mediates the proteasomal targeting of misfolded, mislocalized or accumulated proteins (PubMed:15280365, PubMed:27113755, PubMed:29666234, PubMed:30612738). Acts by binding polyubiquitin chains of target proteins via its UBA domain and by interacting with subunits of the proteasome via its ubiquitin-like domain (PubMed:15280365, PubMed:27113755, PubMed:30612738). Key regulator of DNA repair that represses homologous recombination repair: in response to DNA damage, recruited to sites of DNA damage following phosphorylation by ATM and acts by binding and removing ubiquitinated MRE11 from damaged chromatin, leading to MRE11 degradation by the proteasome (PubMed:30612738). MRE11 degradation prevents homologous recombination repair, redirecting double-strand break repair toward non-homologous end joining (NHEJ) (PubMed:30612738). Specifically recognizes and binds mislocalized transmembrane-containing proteins and targets them to proteasomal degradation (PubMed:27113755). Collaborates with DESI1/POST in the export of ubiquitinated proteins from the nucleus to the cytoplasm (PubMed:29666234). Also plays a role in the regulation of the proteasomal degradation of non-ubiquitinated GJA1 (By similarity). Acts as an adapter protein that recruits UBQLN1 to the autophagy machinery (PubMed:23459205). Mediates the association of UBQLN1 with autophagosomes and the autophagy-related protein LC3 (MAP1LC3A/B/C) and may assist in the maturation of autophagosomes to autolysosomes by mediating autophagosome-lysosome fusion (PubMed:23459205)