Intrinsic protein-protein interaction-mediated and chaperonin-assisted sequential assembly of stable bardet-biedl syndrome protein complex, the BBSome

Q Zhang, D Yu, S Seo, EM Stone… - Journal of Biological …, 2012 - ASBMB
The pleiotropic features of obesity, retinal degeneration, polydactyly, kidney abnormalities,
cognitive impairment, hypertension, and diabetes found in Bardet-Biedl syndrome (BBS)
make this disorder an important model disorder for identifying molecular mechanisms
involved in common human diseases. To date, 16 BBS genes have been reported, seven of
which (BBS1, 2, 4, 5, 7, 8, and 9) code for proteins that form a complex known as the
BBSome. The function of the BBSome involves ciliary membrane biogenesis. Three …