論文 - 詳細
| RRC ID | 46654 |
|---|---|
| 著者 | Miller RM, Portman DS. |
| タイトル | A latent capacity of the C. elegans polycystins to disrupt sensory transduction is repressed by the single-pass ciliary membrane protein CWP-5. |
| ジャーナル | Dis Model Mech |
| Abstract |
Autosomal dominant polycystic kidney disease (ADPKD) results from loss-of-function mutations in PKD1 or PKD2. The products of these genes, the polycystins PC-1 and PC-2, form a transmembrane channel that is necessary for flow sensing by renal cilia. In C. elegans, the polycystin orthologs LOV-1 and PKD-2 function in sensory neurons that mediate male mating behavior. Here, we report that the novel single-pass membrane protein CWP-5 is necessary for polycystin signaling during the response step of mating behavior. As with the polycystins, CWP-5 localizes to neuronal cilia; this localization requires LOV-1. The response defect of cwp-5 mutants does not appear to result from disruption of ciliogenesis or polycystin localization. Instead, genetic and behavioral analyses indicate that CWP-5 represses a previously undescribed antagonistic effect of the polycystins on sensory function. Although cwp-5 does not have a primary-sequence ortholog in vertebrates, it has intriguing parallels with the autosomal recessive PKD gene FPC (also known as PKHD1). Together, this study identifies a new component of C. elegans polycystin signaling, demonstrates that the polycystins have a latent capacity to hinder sensory transduction, and suggests that aberrant functions of the polycystins could contribute to the pathogenesis of PKD. |
| 巻・号 | 3(7-8) |
| ページ | 441-50 |
| 公開日 | 2010-1-1 |
| DOI | 10.1242/dmm.002816 |
| PII | dmm.002816 |
| PMID | 20223935 |
| PMC | PMC2898535 |
| MeSH | Animals Behavior, Animal Caenorhabditis elegans / cytology Caenorhabditis elegans / genetics Caenorhabditis elegans / metabolism* Caenorhabditis elegans Proteins / genetics Caenorhabditis elegans Proteins / metabolism* Cilia / metabolism* Gene Expression Regulation Genes, Helminth Intracellular Membranes / metabolism Male Membrane Proteins / metabolism* Models, Genetic Mutation / genetics Protein Transport Sensory Receptor Cells / metabolism* Sex Characteristics Signal Transduction* TRPP Cation Channels / metabolism* |
| IF | 4.651 |
| 引用数 | 8 |
| WOS 分野 | PATHOLOGY CELL BIOLOGY |
| オルトメトリクス指標 |
オルトメトリクス指標項目
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| 各媒体での言及数の合計 | 0 |
| リソース情報 | |
| 線虫 | tm727 tm1893 |