RRC ID 434
著者 Jauregui AR, Barr MM.
タイトル Functional characterization of the C. elegans nephrocystins NPHP-1 and NPHP-4 and their role in cilia and male sensory behaviors.
ジャーナル Exp Cell Res
Abstract Autosomal dominant polycystic kidney disease (ADPKD) and nephronophthisis (NPH) share two common features: cystic kidneys and ciliary localized gene products. Mutation in either the PKD1 or PKD2 gene accounts for 95% of all ADPKD cases. Mutation in one of four genes (NPHP1-4) results in nephronophthisis. The NPHP1, NPHP2, PKD1, and PKD2 protein products (nephrocystin-1, nephrocystin-2 or inversin, polycystin-1, and polycystin-2, respectively) localize to primary cilia of renal epithelia. However, the relationship between the nephrocystins and polycystins, if any, is unknown. In the nematode Caenorhabditis elegans, the LOV-1 and PKD-2 polycystins localize to male-specific sensory cilia and are required for male mating behaviors. To test the hypothesis that ADPKD and NPH cysts arise from a common defect in cilia, we characterized the C. elegans homologs of NPHP1 and NPHP4. C. elegans nphp-1 and nphp-4 are expressed in a subset of sensory neurons. GFP-tagged NPHP-1 and NPHP-4 proteins localize to ciliated sensory endings of dendrites and colocalize with PKD-2 in male-specific sensory cilia. The cilia of nphp-1(ok500) and nphp-4(tm925) mutants are intact. nphp-1; nphp-4 double, but not single, mutant males are response defective. We propose that NPHP-1 and NPHP-4 proteins play important and redundant roles in facilitating ciliary sensory signal transduction.
巻・号 305(2)
ページ 333-42
公開日 2005-5-1
DOI 10.1016/j.yexcr.2005.01.008
PII S0014-4827(05)00017-0
PMID 15817158
MeSH Alleles Animals Caenorhabditis elegans / physiology* Caenorhabditis elegans Proteins / analysis Caenorhabditis elegans Proteins / genetics Caenorhabditis elegans Proteins / physiology* Cilia / chemistry Cilia / physiology* Green Fluorescent Proteins / analysis Green Fluorescent Proteins / genetics Male Membrane Proteins / analysis Membrane Proteins / metabolism Neurons, Afferent / chemistry Neurons, Afferent / metabolism* Sequence Deletion / genetics Signal Transduction TRPP Cation Channels
IF 3.383
引用数 37
WOS 分野 ONCOLOGY CELL BIOLOGY
リソース情報
線虫 tm925