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Abstract：
The BBSome is an octameric protein complex that regulates ciliary transport and signaling. Mutations in BBSome subunits are closely associated with ciliary defects and lead to ciliopathies, notably Bardet-Biedl syndrome. Over the past few years, there has been significant progress in elucidating the molecular organization and functions of the BBSome complex. An improved understanding of BBSome-mediated biological events and molecular mechanisms is expected to help advance the development of diagnostic and therapeutic approaches for BBSome-related diseases. Here, we review the current literature on the structural assembly, transport regulation, and molecular functions of the BBSome, emphasizing its roles in cilium-related processes. We also provide perspectives on the pathological role of the BBSome in ciliopathies as well as how these can be exploited for therapeutic benefit.
摘要：
BBSome是调节纤毛运输和信号传导的八聚体蛋白复合物。BB中的突变一些亚基与纤毛缺陷密切相关，并导致纤毛病变，特别是Bardet-Biedl综合征。在过去的几年里，在阐明BBSome复合物的分子组织和功能方面取得了重大进展。对BBSome介导的生物事件和分子机制的更好理解有望帮助促进BBSome相关疾病的诊断和治疗方法的发展。这里,我们回顾了目前关于结构装配的文献，运输条例,和BBSome的分子功能，强调其在纤毛相关过程中的作用。我们还提供了关于BBSome在纤毛病中的病理作用的观点，以及如何利用这些治疗益处。
