{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T07:56:20Z","timestamp":1779350180572,"version":"3.51.4"},"reference-count":59,"publisher":"The Company of Biologists","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>The cystic kidney diseases Nephronophthisis (NPHP), Meckel Gruber Syndrome (MKS), and Joubert Syndrome (JBTS) share an underlying etiology of dysfunctional cilia. Patients diagnosed with NPHP type II have mutations in the gene INVS\/NPHP2, which encodes inversin, a cilia localizing protein. Here, we show that the C. elegans inversin ortholog, NPHP-2, localizes to the middle segment of sensory cilia, and is partially redundant with nphp-1 and nphp-4 (orthologs of human nephrocystin-1 and nephrocystin-4, respectively) for cilia placement within the head and tail sensilla. nphp-2 also genetically interacts with MKS ciliopathy gene orthologs, including mks-1, mks-3, mks-6, mksr-1, and mksr-2, in a sensilla-dependent manner to control cilia formation and placement. However, nphp-2 is not required for correct localization of the NPHP and MKS encoded ciliary transition zone proteins or for intraflagellar transport (IFT). We conclude that INVS\/NPHP2 is conserved in C. elegans, and that nphp-2 plays an important role in C. elegans cilia acting as a modifier of the previously described NPHP and MKS pathways to control cilia formation and development.<\/jats:p>","DOI":"10.1242\/jcs.095539","type":"journal-article","created":{"date-parts":[[2012,3,6]],"date-time":"2012-03-06T05:07:03Z","timestamp":1331010423000},"source":"Crossref","is-referenced-by-count":32,"title":["Ciliogenesis in<i>Caenorhabditis elegans<\/i>requires genetic interactions between ciliary middle segment localized NPHP-2 (inversin) and transition zone-associated proteins"],"prefix":"10.1242","author":[{"given":"Simon R. F.","family":"Warburton-Pitt","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrew R.","family":"Jauregui","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chunmei","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Juan","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michel R.","family":"Leroux","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maureen M.","family":"Barr","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"237","published-online":{"date-parts":[[2012,1,1]]},"reference":[{"key":"2021042521474053100_R1","doi-asserted-by":"crossref","first-page":"3389","DOI":"10.1093\/nar\/25.17.3389","article-title":"Gapped BLAST and PSI-BLAST: a new generation of protein database search programs","volume":"25","author":"Altschul","year":"1997","journal-title":"Nucleic Acids Res."},{"key":"2021042521474053100_R2","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1146\/annurev.genom.7.080505.115610","article-title":"The ciliopathies: an emerging class of human genetic disorders","volume":"7","author":"Badano","year":"2006","journal-title":"Annu. 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