Contents

Search


BBsome complex

Function: - required for ciliogenesis - dispensable for centriolar satellite function - ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body & contacts the BBsome Rab8(GTP) enters the primary cilium & promotes extension of the ciliary membrane - 1st the BBsome associates with the ciliary membrane & binds to Rabin8, the guanosyl exchange factor (GEF) for Rab8 & then the Rab8-GTP localizes to the cilium & promotes docking & fusion of carrier vesicles to the base of the ciliary membrane - binds to PCM1 & tubulin Structure: - subunits inlcude BBS1, BBS2, BBS4, BBS5, BBS7, BBS8, BBS9 & BBIP10

General

molecular complex

Properties

SUBUNITS: Bardet-Biedl syndrome 1 protein COMPARTMENT: cytoplasm MOTIF: acetylation site SITE: N-TERMINUS EFFECTOR-BOUND: acetyl Thr phosphorylation site {T369} Thr phosphorylation site {T392} Bardet-Biedl syndrome 2 protein COMPARTMENT: cytoplasm Bardet-Biedl syndrome 4 protein COMPARTMENT: cytoplasm MOTIF: localization to centrosomes {1-66} tetratricopeptide repeat {67-100} PCM1 {101-337} MOTIF: tetratricopeptide repeat {101-134} tetratricopeptide repeat {135-168} tetratricopeptide repeat {169-201} tetratricopeptide repeat {203-235} tetratricopeptide repeat {237-269} tetratricopeptide repeat {270-303} tetratricopeptide repeat {304-337} centrosome {338-519} MOTIF: tetratricopeptide repeat {339-371} tetratricopeptide repeat {373-408} Bardet-Biedl syndrome 5 protein Bardet-Biedl syndrome 7 protein COMPARTMENT: cytoplasm tetratricopeptide repeat protein 8 COMPARTMENT: cytoplasm MOTIF: tetratricopeptide repeat {14-47} tetratricopeptide repeat {251-284} tetratricopeptide repeat {285-317} tetratricopeptide repeat {318-351} tetratricopeptide repeat {352-385} tetratricopeptide repeat {386-419} tetratricopeptide repeat {423-456} tetratricopeptide repeat {457-490} parathyroid horomone responsive B1 protein BBIP10

References

UniProt :accession Q9BXC9

Components

Bardet-Biedl syndrome 1 protein; BBS2-like protein 2 (BBS1, BBS2L2) Bardet-Biedl syndrome 2 protein (BBS2) Bardet-Biedl syndrome 4 protein (BBS4) Bardet-Biedl syndrome 5 protein (BBS5) Bardet-Biedl syndrome 7 protein; BBS2-like protein 1 (BBS7, BBS2L1) parathyroid horomone responsive B1 protein; protein PTHB1; Bardet-Biedl syndrome 9 protein (PTHB1, BBS9) tetratricopeptide repeat protein 8; TPR repeat protein 8; Bardet-Biedl syndrome 8 protein (TTC8, BBS8)