Pericentrin forms a complex with intraflagellar transport proteins and polycystin-2 and is required for primary cilia assembly
نویسندگان
چکیده
Primary cilia are nonmotile microtubule structures that assemble from basal bodies by a process called intraflagellar transport (IFT) and are associated with several human diseases. Here, we show that the centrosome protein pericentrin (Pcnt) colocalizes with IFT proteins to the base of primary and motile cilia. Immunogold electron microscopy demonstrates that Pcnt is on or near basal bodies at the base of cilia. Pcnt depletion by RNA interference disrupts basal body localization of IFT proteins and the cation channel polycystin-2 (PC2), and inhibits primary cilia assembly in human epithelial cells. Conversely, silencing of IFT20 mislocalizes Pcnt from basal bodies and inhibits primary cilia assembly. Pcnt is found in spermatocyte IFT fractions, and IFT proteins are found in isolated centrosome fractions. Pcnt antibodies coimmunoprecipitate IFT proteins and PC2 from several cell lines and tissues. We conclude that Pcnt, IFTs, and PC2 form a complex in vertebrate cells that is required for assembly of primary cilia and possibly motile cilia and flagella.
منابع مشابه
The intraflagellar transport protein IFT20 is associated with the Golgi complex and is required for cilia assembly.
Eukaryotic cilia are assembled via intraflagellar transport (IFT) in which large protein particles are motored along ciliary microtubules. The IFT particles are composed of at least 17 polypeptides that are thought to contain binding sites for various cargos that need to be transported from their site of synthesis in the cell body to the site of assembly in the cilium. We show here that the IFT...
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Eukaryotic cilia are assembled via intraflagellar transport (IFT) in which large protein particles are motored along ciliary microtubules. The IFT particles are composed of at least 17 polypeptides that are thought to contain binding sites for various cargos that need to be transported from their site of synthesis in the cell body to the site of assembly in the cilium. We show here that the IFT...
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Defects in the PKD1 and PKD2 genes cause autosomal dominant polycystic kidney disease (PKD) in ~1 in 1000 adults worldwide. These genes encode polycystin-1 and polycystin-2, which are membrane proteins thought to be involved in a calcium signal transduction cascade that controls epithelial proliferation and differentiation. Individuals with mutations in these genes develop cysts in the ducts an...
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ورودعنوان ژورنال:
- The Journal of Cell Biology
دوره 166 شماره
صفحات -
تاریخ انتشار 2004