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Published online March 3, 2008
doi:10.1083/jcb.200707090
The Journal of Cell Biology, Vol. 180, No. 5, 973-988
The Rockefeller University Press, 0021-9525 $30.00
© 2008 Jauregui et al.
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Article

 The Caenorhabditis elegans nephrocystins act as global modifiers of cilium structure

Andrew R. Jauregui1,2, Ken C.Q. Nguyen4, David H. Hall4, and Maureen M. Barr1,3

1 Department of Genetics, Rutgers University, Piscataway, NJ 08854
2 Laboratory of Genetics, and 3 School of Pharmacy, University of Wisconsin-Madison, Madison, WI 53705
4 Center for C. elegans Anatomy, Department of Neuroscience, Albert Einstein College of Medicine, New York, NY 10461

Correspondence to M.M. Barr: barr{at}biology.rutgers.edu

Nephronophthisis (NPHP) is the most common genetic cause of end-stage renal disease in children and young adults. In Chlamydomonas reinhardtii, Caenorhabditis elegans, and mammals, the NPHP1 and NPHP4 gene products nephrocystin-1 and nephrocystin-4 localize to basal bodies or ciliary transition zones (TZs), but their function in this location remains unknown. We show here that loss of C. elegans NPHP-1 and NPHP-4 from TZs is tolerated in developing cilia but causes changes in localization of specific ciliary components and a broad range of subtle axonemal ultrastructural defects. In amphid channel cilia, nphp-4 mutations cause B tubule defects that further disrupt intraflagellar transport (IFT). We propose that NPHP-1 and NPHP-4 act globally at the TZ to regulate ciliary access of the IFT machinery, axonemal structural components, and signaling molecules, and that perturbing this balance results in cell type–specific phenotypes.

Abbreviations used in this paper: BBS, Bardet-Biedl syndrome; Dyf, dye filling defective; IFT, intraflagellar transport; NPHP, nephronophthisis; PKD, polycystic kidney disease; SynDyf, synthetic Dyf; TAM, tubulin-associated material; TEM, transmission EM; TRP, transient receptor potential; TRPP, TRP polycystin; TRPV, TRP vanilloid; TZ, transition zone.


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