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The C-elegans homologs of nephrocystin-1 and nephrocystin-4 are cilia transition zone proteins involved in chemosensory perception
Södertörns högskola, Institutionen för livsvetenskaper.ORCID-id: 0000-0001-6416-8572
Vise andre og tillknytning
2005 (engelsk)Inngår i: Journal of Cell Science, ISSN 0021-9533, E-ISSN 1477-9137, Vol. 118, nr 23, s. 5575-5587Artikkel i tidsskrift (Fagfellevurdert) Published
Abstract [en]

Nephronophthisis (NPH) is a cystic kidney disorder that causes end-stage renal failure in children. Five nephrocystin (nephrocystin-1 to nephrocystin-5) genes, whose function is disrupted in NPH patients, have been identified and data indicate they form a complex at cell junctions and focal adhesions. More recently, the nephrocystin proteins have also been identified in cilia, as have multiple other cystic kidney disease related proteins. Significant insights into this cilia and cystic kidney disease connection have come from analyses in simpler eukaryotic organisms such as Caenorhabditis elegans. In this regard, we became interested in the C elegans homologs of nephrocystin-1 (nph-1) and nephrocystin-4 (nph-4) from a database screen to identify genes coordinately regulated by the ciliogenic transcription factor DAF-19. Here we show that expression of nph-1 and nph-4 is DAF-19 dependent, that their expression is restricted to ciliated sensory neurons, and that both NPH-1 and NPH-4 concentrate at the transition zones at the base of the cilia, but are not found in the cilium axoneme. In addition, NPH-4 is required for the localization of NPH-1 to this domain. Interestingly, nph-1 or nph-4 mutants have no obvious cilia assembly defects; however, they do have abnormalities in cilia-mediated sensory functions as evidenced by abnormal chemotaxis and lifespan regulation. Our data suggest that rather than having a ciliogenic role, the NPH proteins play an important function as part of the sensory or signaling machinery of this organelle. These findings suggest that the defects in human NPH patients may not be the result of aberrant ciliogenesis but abnormal cilia-sensory functions.

sted, utgiver, år, opplag, sider
2005. Vol. 118, nr 23, s. 5575-5587
HSV kategori
Identifikatorer
URN: urn:nbn:se:sh:diva-14434DOI: 10.1242/jcs.02665ISI: 000234413200016PubMedID: 16291722Scopus ID: 2-s2.0-30544454640OAI: oai:DiVA.org:sh-14434DiVA, id: diva2:487468
Tilgjengelig fra: 2012-01-31 Laget: 2011-12-23 Sist oppdatert: 2018-07-18bibliografisk kontrollert

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