論文 - 詳細
| RRC ID | 45658 |
|---|---|
| 著者 | Xu Q, Zhang Y, Wei Q, Huang Y, Li Y, Ling K, Hu J. |
| タイトル | BBS4 and BBS5 show functional redundancy in the BBSome to regulate the degradative sorting of ciliary sensory receptors. |
| ジャーナル | Sci Rep |
| Abstract |
Cilia harbor sensory receptors for various signaling cascades critical for vertebrate development. However, the mechanisms underlying the ciliary homeostasis of sensory receptors remain elusive. Here, we demonstrate that BBS-4 and BBS-5, two distinct BBSome components, show unexpected functional redundancy in the context of cilia in C. elegans. BBS-4 directly interacts with BBS-5 and the interaction can be disrupted by a conserved mutation identified in human BBS4. Surprisingly, we found that BBS-4 and BBS-5 act redundantly in the BBSome to regulate the ciliary removal, rather than the ciliary entry or retrograde IFT transport, of various sensory receptors. Further analyses indicate that co-depletion of BBS-4 and BBS-5 disrupts the lysosome-targeted degradative sorting of ciliary sensory receptors. Moreover, mammalian BBS4 and BBS5 also interact directly and coordinate the ciliary removal of polycystin 2. Hence, we reveal a novel and highly conserved role for the BBSome in fine-tuning ciliary signaling by regulating the ciliary removal of sensory receptors for lysosomal degradation. |
| 巻・号 | 5 |
| ページ | 11855 |
| 公開日 | 2015-7-7 |
| DOI | 10.1038/srep11855 |
| PII | srep11855 |
| PMID | 26150102 |
| PMC | PMC4493597 |
| MeSH | Animals Bardet-Biedl Syndrome / metabolism Bardet-Biedl Syndrome / pathology Caenorhabditis elegans Caenorhabditis elegans Proteins / antagonists & inhibitors Caenorhabditis elegans Proteins / genetics Caenorhabditis elegans Proteins / metabolism* Cell Line Cilia / metabolism* Cytoskeletal Proteins Down-Regulation HEK293 Cells Humans Microtubule-Associated Proteins Mutation, Missense Phosphate-Binding Proteins Proteins / antagonists & inhibitors Proteins / genetics Proteins / metabolism* RNA Interference RNA, Small Interfering / metabolism Sensory Receptor Cells / metabolism* Ubiquitination |
| IF | 3.998 |
| 引用数 | 33 |
| WOS 分野 | 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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| 最多言及媒体 | X(Twitter) |
| 各媒体での言及数の合計 | 3 |
| 過去6か月間でのオルトメトリクス指標の変動値 | 0.0 |
| リソース情報 | |
| 線虫 | tm3038 |