Bardet-Biedl syndrome 5 protein
1_MSVLD 6_ ALWED 11_ RDVRF 16_ DLSAQ 21_ QMKTR 26_ PGEVL 31_ IDCLD 36_ SIEDT 41_ KGNNG 46_ DRGRL 51_ LVTNL 56_ RILWH 61_ SLALS 66_ RVNVS 71_ VGYNC 76_ ILNIT 81_ TRTAN 86_ SKLRG 91_ QTEAL 96_ YILTK 101_ CNSTR 106_ FEFIF 111_ TNLVP 116_ GSPRL 121_ FTSVM 126_ AVHRA 131_ YETSK 136_ MYRDF 141_ KLRSA 146_ LIQNK 151_ QLRLL 156_ PQEHV 161_ YDKIN 166_ GVWNL 171_ SSDQG 176_ NLGTF 181_ FITNV 186_ RIVWH 191_ ANMND 196_ SFNVS 201_ IPYLQ 206_ IRSIK 211_ IRDSK 216_ FGLAL 221_ VIESS 226_ QQSGG 231_ YVLGF 236_ KIDPV 241_ EKLQE 246_ SVKEI 251_ NSLHK 256_ VYSAS 261_ PIFGV 266_ DYEME 271_ EKPQP 276_ LEALT 281_ VEQIQ 286_ DDVEI 291_ DSDGH 296_ TDAFV 301_ AYFAD 306_ GNKQQ 311_ DREPV 316_ FSEEL 321_ GLAIE 326_ KLKDG 331_ FTLQG 336_LWEVM
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 BBSome complex ciliary localization but not for the proper complex assembly