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Deletion of IFT20 in the mouse kidney causes misorientation of the mitotic spindle and cystic kidney disease

¹ Program in Molecular Medicine, University of Massachusetts Medical School, Worcester, MA 01605
² Department of Physiology, University of Massachusetts Medical School, Worcester, MA 01655

Correspondence to Gregory J. Pazour: gregory.pazour{at}umassmed.edu

Primary cilia project from the surface of most vertebrate cells and are thought to be sensory organelles. Defects in primary cilia lead to cystic kidney disease, although the ciliary mechanisms that promote and maintain normal renal function remain incompletely understood. In this work, we generated a floxed allele of the ciliary assembly gene Ift20. Deleting this gene specifically in kidney collecting duct cells prevents cilia formation and promotes rapid postnatal cystic expansion of the kidney. Dividing collecting duct cells in early stages of cyst formation fail to properly orient their mitotic spindles along the tubule, whereas nondividing cells improperly position their centrosomes. At later stages, cells lacking cilia have increased canonical Wnt signaling and increased rates of proliferation. Thus, IFT20 functions to couple extracellular events to cell proliferation and differentiation.

Abbreviations used in this paper: DBA, Dolichos biflorus agglutinin; e, embryonic day; GAPDH, glyceraldehyde 3-phosphate dehydrogenase; HNF, hepatocyte nuclear factor; IFT, intraflagellar transport; p, postnatal day; PKD, polycystic kidney disease; qPCR, quantitative real-time PCR; Tcf, T cell factor.

© 2008 Jonassen et al. This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.jcb.org/misc/terms.shtml). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 3.0 Unported license, as described at http://creativecommons.org/licenses/by-nc-sa/3.0/).

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