In previous work, we identified a stable complex (BBSome) consisting of eight proteins that binds PCM-1 and tubulin. Our data indicate that the BBSome performs an essential ciliogenic function at the basal body and inside the cilium. The cilia-related function of the BBSome is mediated, at least in part, through the Rab8 GDP/GTP exchange factor (Rabin8). The relationship between the BBSome and other BBS proteins was unknown.
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January 26th, 2010
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March 3rd, 2008
To dissect the mechanisms involved in the metabolic disorders associated with BBS, we assessed the development of obesity in multiple BBS mouse models developed in our laboratory. We found that BBS mice are hyperphagic, have low motor activity, and elevated circulating levels of leptin. The effect of exogenous leptin on body weight and food intake is attenuated in BBS mice, suggesting that leptin resistance contributes to hyperleptinemia and obesity in these mice.