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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Kida, Katarzyna Chen, Nansheng Blacque, Oliver E. Yoder, Bradley K. Leroux, Michel R. Li, Chunmei Williams, Corey L. Stupay, Rachel M. Bialas, Nathan J. Semenec, Lucie Inglis, Peter N. Mohan, Swetha |
| Description | Author Affiliation: Williams CL ( Department of Cell Biology, University of Alabama, Birmingham, AL 35294, USA.) |
| Abstract | Meckel-Gruber syndrome (MKS), nephronophthisis (NPHP), and related ciliopathies present with overlapping phenotypes and display considerable allelism between at least twelve different genes of largely unexplained function. We demonstrate that the conserved C. elegans B9 domain (MKS-1, MKSR-1, and MKSR-2), MKS-3/TMEM67, MKS-5/RPGRIP1L, MKS-6/CC2D2A, NPHP-1, and NPHP-4 proteins exhibit essential, collective functions at the transition zone (TZ), an underappreciated region at the base of all cilia characterized by Y-shaped assemblages that link axoneme microtubules to surrounding membrane. These TZ proteins functionally interact as members of two distinct modules, which together contribute to an early ciliogenic event. Specifically, MKS/MKSR/NPHP proteins establish basal body/TZ membrane attachments before or coinciding with intraflagellar transport–dependent axoneme extension and subsequently restrict accumulation of nonciliary components within the ciliary compartment. Together, our findings uncover a unified role for eight TZ-localized proteins in basal body anchoring and establishing a ciliary gate during ciliogenesis, and suggest that disrupting ciliary gate function contributes to phenotypic features of the MKS/NPHP disease spectrum. |
| ISSN | 00219525 |
| e-ISSN | 15408140 |
| Journal | The Journal of Cell Biology |
| Issue Number | 6 |
| Volume Number | 192 |
| Language | English |
| Publisher | Rockefeller University Press (United States) |
| Publisher Date | 2011-03-21 |
| Publisher Place | United States |
| Access Restriction | Open |
| Subject Keyword | Caenorhabditis Elegans Proteins Metabolism Physiology Ultrastructure Membrane Proteins Animals Caenorhabditis Elegans Genetics Ciliary Motility Disorders Pathology Physiopathology Encephalocele Kidney Diseases, Cystic Congenital Polycystic Kidney Diseases Protein Isoforms Recombinant Fusion Proteins Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Cell Biology |
| Content Type | Text |
| Resource Type | Article |
| Subject | Cell Biology Medicine |
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