| Primary Identifier | IPR026511 | Type | Family |
| Short Name | PTHB1 |
| description | Parathyroid hormone-responsive B1 (PTHB1, also known as BBS9 in human) is part of the BBSome complex. The BBSome complex (contains BBS1, BBS2, BBS4, BBS5, BBS7, BBS8, BBS9 and BBIP10) 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 localises 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 []. |
