Polaris, a protein disrupted in orpk mutant mice, is required for assembly of renal cilium
- PMID: 11832437
- DOI: 10.1152/ajprenal.00273.2001
Polaris, a protein disrupted in orpk mutant mice, is required for assembly of renal cilium
Abstract
Cilia are organelles that play diverse roles, from fluid movement to sensory reception. Polaris, a protein associated with cystic kidney disease in Tg737(o)(rpk) mice, functions in a ciliogenic pathway. Here, we explore the role of polaris in primary cilia on Madin-Darby canine kidney cells. The results indicate that polaris localization and solubility change dramatically during cilia formation. These changes correlate with the formation of basal bodies and large protein rafts at the apical surface of the epithelia. A cortical collecting duct cell line has been derived from mice with a mutation in the Tg737 gene. These cells do not develop normal cilia, which can be corrected by reexpression of the wild-type Tg737 gene. These data suggest that the primary cilia are important for normal renal function and/or development and that the ciliary defect may be a contributing factor to the cystic disease in Tg737(o)(rpk) mice. Further characterization of these cells will be important in elucidating the physiological role of renal cilia and in determining their relationship to cystic disease.
Similar articles
-
Delayed cystogenesis and increased ciliogenesis associated with the re-expression of polaris in Tg737 mutant mice.Kidney Int. 2003 Apr;63(4):1220-9. doi: 10.1046/j.1523-1755.2003.00863.x. Kidney Int. 2003. PMID: 12631338
-
The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia.J Am Soc Nephrol. 2002 Oct;13(10):2508-16. doi: 10.1097/01.asn.0000029587.47950.25. J Am Soc Nephrol. 2002. PMID: 12239239
-
The C. elegans homolog of the murine cystic kidney disease gene Tg737 functions in a ciliogenic pathway and is disrupted in osm-5 mutant worms.Development. 2001 May;128(9):1493-505. doi: 10.1242/dev.128.9.1493. Development. 2001. PMID: 11290289
-
Putative roles of cilia in polycystic kidney disease.Biochim Biophys Acta. 2011 Oct;1812(10):1256-62. doi: 10.1016/j.bbadis.2011.04.012. Epub 2011 May 8. Biochim Biophys Acta. 2011. PMID: 21586324 Review.
-
Polycystic kidney disease and the renal cilium.Nephrology (Carlton). 2007 Dec;12(6):559-64. doi: 10.1111/j.1440-1797.2007.00869.x. Nephrology (Carlton). 2007. PMID: 17995581 Review.
Cited by
-
Primary cilia control glucose homeostasis via islet paracrine interactions.Proc Natl Acad Sci U S A. 2020 Apr 21;117(16):8912-8923. doi: 10.1073/pnas.2001936117. Epub 2020 Apr 6. Proc Natl Acad Sci U S A. 2020. PMID: 32253320 Free PMC article.
-
Architecture and function of IFT complex proteins in ciliogenesis.Differentiation. 2012 Feb;83(2):S12-22. doi: 10.1016/j.diff.2011.11.001. Epub 2011 Nov 25. Differentiation. 2012. PMID: 22118932 Free PMC article. Review.
-
Mouse models of polycystic kidney disease induced by defects of ciliary proteins.BMB Rep. 2013 Feb;46(2):73-9. doi: 10.5483/bmbrep.2013.46.2.022. BMB Rep. 2013. PMID: 23433108 Free PMC article. Review.
-
Cilia proteins getting to work - how do they commute from the cytoplasm to the base of cilia?J Cell Sci. 2022 Sep 1;135(17):jcs259444. doi: 10.1242/jcs.259444. Epub 2022 Sep 8. J Cell Sci. 2022. PMID: 36073764 Free PMC article. Review.
-
Knockdown of bicaudal C in zebrafish (Danio rerio) causes cystic kidneys: a nonmammalian model of polycystic kidney disease.Comp Med. 2010 Apr;60(2):96-106. Comp Med. 2010. PMID: 20412683 Free PMC article.
Publication types
MeSH terms
Substances
Grants and funding
LinkOut - more resources
Full Text Sources
Molecular Biology Databases