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Autosomal dominant polycystic kidney disease (ADPKD), a common cause of kidney failure, results from mutations in PKD1 or PKD2, encoding polycystin-1 and polycystin-2. Here, we establish C. elegans as a platform for the functional classification of PC2 variants by characterizing PKD-2C180S, the C. elegans ortholog of the likely pathogenic human variant PC2C331S. Using CRISPR\/Cas9 genome editing with dual-color fluorescent reporters and super-resolution imaging, we show that PKD-2C180S reduces cell-body protein levels, abolishes ciliary and extracellular vesicle (EV) localization, and eliminates sensory function, phenocopying a pkd-2 null allele. In heterozygous animals, PKD-2C180S is recessive and exerts no dominant-negative effect on wild-type PKD-2 trafficking, protein levels, or function, establishing that PKD-2 is haplosufficient in this model. PKD-2C180S also abolishes ciliary and EV localization of the PC1 homolog LOV-1 and reduces LOV-1 cell-body levels, phenocopying pkd-2 null animals, consistent with PC2 acting as a molecular chaperone for PC1 stability and trafficking. Genetic epistasis analysis shows that PKD-2C180S protein levels and ciliary trafficking defects are intrinsic to the mutant protein and independent of lov-1 and the ciliary kinesin-3 klp-6. Quantitative analysis reveals that LOV-1\u2022PKD-2 is more abundant at the ciliary membrane and more efficiently packaged into EVs than PKD-2 alone in lov-1 mutants. We conclude that PC2C331S may perturb protein stability and\/or polycystin complex formation prior to ciliary\/EV trafficking. This work establishes a C. elegans pipeline for mechanistic classification of conserved ADPKD-associated missense variants.<\/jats:p>","DOI":"10.1093\/genetics\/iyag182","type":"journal-article","created":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T20:59:12Z","timestamp":1783976352000},"source":"Crossref","is-referenced-by-count":0,"title":["A\n                    <i>C. elegans<\/i>\n                    model for functional analysis of conserved ADPKD variants in cilia, extracellular vesicles, and sensory signaling"],"prefix":"10.1093","author":[{"given":"Juan","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Genetics and Human Genetics Institute of New Jersey, Rutgers, The State University of New Jersey , Piscataway, NJ 08854 ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Nava Cruz","sequence":"additional","affiliation":[{"name":"Department of Genetics and Human Genetics Institute of New Jersey, Rutgers, The State University of New Jersey , Piscataway, NJ 08854 ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathon D","family":"Walsh","sequence":"additional","affiliation":[{"name":"Department of Genetics and Human Genetics Institute of New Jersey, Rutgers, The State University of New Jersey , Piscataway, NJ 08854 ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"desRanleau","sequence":"additional","affiliation":[{"name":"Department of Genetics and Human Genetics Institute of New Jersey, Rutgers, The State University of New Jersey , Piscataway, NJ 08854 ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8498-848X","authenticated-orcid":false,"given":"Inna A","family":"Nikonorova","sequence":"additional","affiliation":[{"name":"Department of Genetics and Human Genetics Institute of New Jersey, Rutgers, The State University of New Jersey , Piscataway, NJ 08854 ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4483-2952","authenticated-orcid":false,"given":"Maureen M","family":"Barr","sequence":"additional","affiliation":[{"name":"Department of Genetics and Human Genetics Institute of New Jersey, Rutgers, The State University of New Jersey , Piscataway, NJ 08854 ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,7,13]]},"reference":[{"key":"2026092414440578400_iyag182-B1","doi-asserted-by":"publisher","first-page":"493","DOI":"10.1038\/s41586-024-07487-w","article-title":"Accurate structure prediction of biomolecular interactions with AlphaFold 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