The Caenorhabditis elegans nephrocystins act as global modifiers of cilium structure

Andrew R. Jauregui, Ken C.Q. Nguyen, David H. Hall, Maureen M. Barr

Research output: Contribution to journalArticle

78 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)973-988
Number of pages16
JournalJournal of Cell Biology
Volume180
Issue number5
DOIs
StatePublished - Mar 10 2008

ASJC Scopus subject areas

  • Cell Biology

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