BBSome


The BBSome is an octameric protein complex. It is a component of the basal body and is involved in trafficking cargos to the primary cilium. The BBSome is a complex of seven Bardet–Biedl syndrome proteins: BBS1, BBS2, BBS4, BBS5, BBS7, BBS8 and BBS9. In addition the BBSome contains the BBIP10 protein. Mutation in each of this eight BBSome genes causes a severe multiorganic syndrome presenting in most cases by retinal dystrophy, obesity, renal anomalies, post-axial polydactyly, and developmental delay.

History

The BBSome was first identified in 2007 by Peter K. Jackson and colleagues.

Assembly

BBSome assembly has been shown to be mediated by a complex containing a further three BBS proteins: BBS6, BBS10 and BBS12. In addition chaperonins of the CCT/TRiC family are involved.