A Core Complex of BBS Proteins Cooperates with the GTPase Rab8 to Promote Ciliary Membrane Biogenesis
Maxence V. Nachury,Alexander V. Loktev,Qihong Zhang,Christopher J. Westlake,Johan Peränen,Andreas Merdes,Diane C. Slusarski,Richard H. Scheller,J. Fernando Bazan,Val C. Sheffield,Peter K. Jackson +10 more
TLDR
The data reveal that BBS may be caused by defects in vesicular transport to the cilium, and a complex composed of seven highly conserved BBS proteins is identified, the BBSome, which localizes to nonmembranous centriolar satellites in the cytoplasm but also to the membrane of the cILium.About:
This article is published in Cell.The article was published on 2007-06-15 and is currently open access. It has received 1299 citations till now. The article focuses on the topics: BBSome & Ciliary base.read more
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The primary cilium: a signalling centre during vertebrate development
TL;DR: The connections between cilia and developmental signalling have begun to clarify the basis of human diseases associated with ciliary dysfunction, and the cilium represents a nexus for signalling pathways during development.
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Role of Rab GTPases in membrane traffic and cell physiology.
Alex H. Hutagalung,Peter Novick +1 more
TL;DR: This review discusses how Rabs can regulate virtually all steps of membrane traffic from the formation of the transport vesicle at the donor membrane to its fusion at the target membrane.
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When cilia go bad: cilia defects and ciliopathies
TL;DR: Cilia — tiny hair-like organelles attached to the cell surface — are located on almost all polarized cell types of the human body and have been adapted as versatile tools for various cellular functions, explaining why cilia-related disorders can affect many organ systems.
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Genes and molecular pathways underpinning ciliopathies
TL;DR: Investigating ciliopathies has helped to understand the molecular mechanisms by which the cilium-associated basal body functions in early ciliogenesis, as well as how the transition zone functions in ciliary gating, and how intraflagellar transport enables cargo trafficking and signalling.
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Ciliogenesis: building the cell's antenna
TL;DR: As cilia are important in sensing and processing developmental signals and directing the flow of fluids such as mucus, defects in ciliogenesis and length control are likely to underlie a range of cilium-related human diseases.
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