دورية أكاديمية
The centriolar satellite proteins Cep72 and Cep290 interact and are required for recruitment of BBS proteins to the cilium.
| العنوان: | The centriolar satellite proteins Cep72 and Cep290 interact and are required for recruitment of BBS proteins to the cilium. |
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| المؤلفون: | Stowe TR; Department of Biology, Stanford School of Medicine, Stanford, CA 94305-5020, USA., Wilkinson CJ, Iqbal A, Stearns T |
| المصدر: | Molecular biology of the cell [Mol Biol Cell] 2012 Sep; Vol. 23 (17), pp. 3322-35. Date of Electronic Publication: 2012 Jul 05. |
| نوع المنشور: | Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S. |
| اللغة: | English |
| بيانات الدورية: | Publisher: American Society for Cell Biology Country of Publication: United States NLM ID: 9201390 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1939-4586 (Electronic) Linking ISSN: 10591524 NLM ISO Abbreviation: Mol Biol Cell Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: Bethesda, MD : American Society for Cell Biology, c1992- |
| مواضيع طبية MeSH: | Cilia/*metabolism , Microtubule-Associated Proteins/*metabolism , Proteins/*metabolism , Zebrafish Proteins/*metabolism, 3T3 Cells ; Animals ; Bardet-Biedl Syndrome ; Cell Cycle Proteins/genetics ; Cell Cycle Proteins/metabolism ; Cell Line ; Centrioles/genetics ; Centrioles/metabolism ; Centrosome ; Humans ; Mice ; Microtubule-Associated Proteins/genetics ; RNA Interference ; RNA, Small Interfering ; Zebrafish ; Zebrafish Proteins/genetics |
| مستخلص: | Defects in centrosome and cilium function are associated with phenotypically related syndromes called ciliopathies. Centriolar satellites are centrosome-associated structures, defined by the protein PCM1, that are implicated in centrosomal protein trafficking. We identify Cep72 as a PCM1-interacting protein required for recruitment of the ciliopathy-associated protein Cep290 to centriolar satellites. Loss of centriolar satellites by depletion of PCM1 causes relocalization of Cep72 and Cep290 from satellites to the centrosome, suggesting that their association with centriolar satellites normally restricts their centrosomal localization. We identify interactions between PCM1, Cep72, and Cep290 and find that disruption of centriolar satellites by overexpression of Cep72 results in specific aggregation of these proteins and the BBSome component BBS4. During ciliogenesis, BBS4 relocalizes from centriolar satellites to the primary cilium. This relocalization occurs normally in the absence of centriolar satellites (PCM1 depletion) but is impaired by depletion of Cep290 or Cep72, resulting in defective ciliary recruitment of the BBSome subunit BBS8. We propose that Cep290 and Cep72 in centriolar satellites regulate the ciliary localization of BBS4, which in turn affects assembly and recruitment of the BBSome. Finally, we show that loss of centriolar satellites in zebrafish leads to phenotypes consistent with cilium dysfunction and analogous to those observed in human ciliopathies. |
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| معلومات مُعتمدة: | R01 GM052022 United States GM NIGMS NIH HHS; GM52022 United States GM NIGMS NIH HHS |
| المشرفين على المادة: | 0 (BBS4 protein, human) 0 (CEP290 protein, zebrafish) 0 (CEP72 protein, human) 0 (Cell Cycle Proteins) 0 (Microtubule-Associated Proteins) 0 (Pcm1 protein, mouse) 0 (Proteins) 0 (RNA, Small Interfering) 0 (Zebrafish Proteins) |
| تواريخ الأحداث: | Date Created: 20120707 Date Completed: 20130222 Latest Revision: 20211021 |
| رمز التحديث: | 20231215 |
| مُعرف محوري في PubMed: | PMC3431927 |
| DOI: | 10.1091/mbc.E12-02-0134 |
| PMID: | 22767577 |
| قاعدة البيانات: | MEDLINE |
| تدمد: | 1939-4586 |
|---|---|
| DOI: | 10.1091/mbc.E12-02-0134 |