Bardet-Biedl syndrome 4 protein
1_MAEER 6_ VATRT 11_ QFPVS 16_ TESQK 21_ PRQKK 26_ APEFP 31_ ILEKQ 36_ NWLIH 41_ LHYIR 46_ KDYEA 51_ CKAVI 56_ KEQLQ 61_ ETQGL 66_ CEYAI 71_ YVQAL 76_ IFRLE 81_ GNIQE 86_ SLELF 91_ QTCAV 96_ LSPQS 101_ ADNLK 106_ QVARS 111_ LFLLG 116_ KHKAA 121_ IEVYN 126_ EAAKL 131_ NQKDW 136_ EISHN 141_ LGVCY 146_ IYLKQ 151_ FNKAQ 156_ DQLHN 161_ ALNLN 166_ RHDLT 171_ YIMLG 176_ KIHLL 181_ EGDLD 186_ KAIEV 191_ YKKAV 196_ EFSPE 201_ NTELL 206_ TTLGL 211_ LYLQL 216_ GIYQK 221_ AFEHL 226_ GNALT 231_ YDPTN 236_ YKAIL 241_ AAGSM 246_ MQTHG 251_ DFDVA 256_ LTKYR 261_ VVACA 266_ VPESP 271_ PLWNN 276_ IGMCF 281_ FGKKK 286_ YVAAI 291_ SCLKR 296_ ANYLA 301_ PFDWK 306_ ILYNL 311_ GLVHL 316_ TMQQY 321_ ASAFH 326_ FLSAA 331_ INFQP 336_ KMGEL 341_ YMLLA 346_ VALTN 351_ LEDIE 356_ NAKRA 361_ YAEAV 366_ HLDKC 371_ NPLVN 376_ LNYAV 381_ LLYNQ 386_ GEKKN 391_ ALAQY 396_ QEMEK 401_ KVSLL 406_ KDNSS 411_ LEFDS 416_ EMVEM 421_ AQKLG 426_ AALQV 431_ GEALV 436_ WTKPV 441_ KDPKS 446_ KHQTT 451_ STSKP 456_ ASFQQ 461_ PLGSN 466_ QALGQ 471_ AMSSA 476_ AAYRT 481_ LPSGA 486_ GGTSQ 491_ FTKPP 496_ SLPLE 501_ PEPAV 506_ ESSPT 511_ETSEQ
1: The BBSome complex is thought to function as a coat complex required for sorting of specific membrane proteins to the primary cilia. The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Firstly the BBSome associates with the ciliary membrane and binds to RAB3IP/Rabin8, the guanosyl exchange factor (GEF) for Rab8 and then the Rab8-GTP localizes to the cilium and promotes docking and fusion of carrier vesicles to the base of the ciliary membrane. The BBSome complex, together with the LTZL1, controls SMO ciliary trafficking and contributes to the sonic hedgehog (SHH) pathway regulation. Required for proper BBSome complex assembly and its ciliary localization. Required for microtubule anchoring at the centrosome but not for microtubule nucleation. May be required for the dynein-mediated transport of pericentriolar proteins to the centrosome