Article
BBS6, BBS10, and BBS12 form a complex with CCT/TRiC family chaperonins and mediate BBSome assembly.
Proceedings of the National Academy of Sciences of the United States of America - 26 Jan 2010
Seo Seongjin, Baye Lisa M, Schulz Nathan P, Beck John S, Zhang Qihong, Slusarski Diane C, Sheffield Val C
Abstract excerpt
Bardet-Biedl syndrome (BBS) is a human genetic disorder resulting in obesity, retinal degeneration, polydactyly, and nephropathy. Recent studies indicate that trafficking defects to the ciliary membrane are involved in this syndrome. Here, we show that a novel complex composed of three chaperonin-like BBS proteins (BBS6, BBS10, and BBS12) and CCT/TRiC family chaperonins mediates BBSome assembly, which transports...
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