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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"}],"commentType":"FUNCTION"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"16327777"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"17574030"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"22072986"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"24939912"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"33144677"}],"value":"Part of BBSome complex, that contains BBS1, BBS2, BBS4, BBS5, BBS7, BBS8/TTC8, BBS9 and BBIP10. Binds to phosphoinositides. Interacts with CCDC28B. Interacts with SMO; the interaction is indicative for the association of SMO with the BBsome complex to facilitate ciliary localization of SMO. Interacts with PKD1 (PubMed:24939912). Interacts with DLEC1 (PubMed:33144677)"}],"commentType":"SUBUNIT"},{"commentType":"INTERACTION","interactions":[{"interactantOne":{"uniProtKBAccession":"Q8N3I7","intActId":"EBI-2892592"},"interactantTwo":{"uniProtKBAccession":"Q3SYG4","geneName":"BBS9","intActId":"EBI-2826852"},"numberOfExperiments":9,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q8N3I7","intActId":"EBI-2892592"},"interactantTwo":{"uniProtKBAccession":"P78560","geneName":"CRADD","intActId":"EBI-520375"},"numberOfExperiments":3,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q8N3I7","intActId":"EBI-2892592"},"interactantTwo":{"uniProtKBAccession":"Q15051","geneName":"IQCB1","intActId":"EBI-2805823"},"numberOfExperiments":8,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"Q8N3I7","intActId":"EBI-2892592"},"interactantTwo":{"uniProtKBAccession":"Q6P597","geneName":"KLC3","intActId":"EBI-1643885"},"numberOfExperiments":3,"organismDiffer":false}]},{"commentType":"SUBCELLULAR LOCATION","note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000250"}],"value":"Localizes to basal bodies"}]},"subcellularLocations":[{"location":{"value":"Cell projection, cilium membrane","id":"SL-0305"}},{"location":{"value":"Cytoplasm","id":"SL-0086"}},{"location":{"evidences":[{"evidenceCode":"ECO:0000250"}],"value":"Cytoplasm, cytoskeleton, cilium basal body","id":"SL-0087"}},{"location":{"value":"Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite","id":"SL-0485"}}]},{"commentType":"ALTERNATIVE PRODUCTS","events":["Alternative splicing"],"isoforms":[{"name":{"value":"1"},"isoformIds":["Q8N3I7-1"],"isoformSequenceStatus":"Displayed"},{"name":{"value":"2"},"isoformIds":["Q8N3I7-2"],"sequenceIds":["VSP_017240"],"isoformSequenceStatus":"Described"}]},{"commentType":"DISEASE","disease":{"diseaseId":"Bardet-Biedl syndrome 5","diseaseAccession":"DI-00163","acronym":"BBS5","description":"A syndrome characterized by usually severe pigmentary retinopathy, early-onset obesity, polydactyly, hypogenitalism, renal malformation and intellectual disability. 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