{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T20:02:30Z","timestamp":1786132950015,"version":"build-2736575974"},"reference-count":50,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"9","funder":[{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"publisher","award":["2022R1A2C3002899"],"award-info":[{"award-number":["2022R1A2C3002899"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"publisher","award":["2020R1I1A1A01072944"],"award-info":[{"award-number":["2020R1I1A1A01072944"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"publisher","award":["2016R1A5A1011974"],"award-info":[{"award-number":["2016R1A5A1011974"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["JASN"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      Ciliogenesis-associated kinase 1 (\n                      <jats:italic toggle=\"yes\">CILK1<\/jats:italic>\n                      ) is a ciliary gene that localizes in primary cilia and regulates ciliary transport. Mutations in\n                      <jats:italic toggle=\"yes\">CILK1<\/jats:italic>\n                      cause various ciliopathies. However, the pathogenesis of CILK1-deficient kidney disease is unknown.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      To examine whether CILK1 deficiency causes PKD accompanied by abnormal cilia, we generated mice with deletion of\n                      <jats:italic toggle=\"yes\">Cilk1<\/jats:italic>\n                      in cells of the renal collecting duct. A yeast two-hybrid system and coimmunoprecipitation (co-IP) were used to identify a novel regulator, kinesin light chain\u20133 (KLC3), of ciliary trafficking and cyst progression in the\n                      <jats:italic toggle=\"yes\">Cilk1<\/jats:italic>\n                      -deficient model. Immunocytochemistry and co-IP were used to examine the effect of KLC3 on ciliary trafficking of the IFT-B complex and EGFR. We evaluated the effects of these genes on ciliary trafficking and cyst progression by modulating CILK1 and KLC3 expression levels.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>CILK1 deficiency leads to PKD accompanied by abnormal ciliary trafficking. KLC3 interacts with CILK1 at cilia bases and is increased in cyst-lining cells of CILK1-deficient mice. KLC3 overexpression promotes ciliary recruitment of IFT-B and EGFR in the CILK1 deficiency condition, which contributes to the ciliary defect in cystogenesis. Reduction in KLC3 rescued the ciliary defects and inhibited cyst progression caused by CILK1 deficiency.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Our findings suggest that CILK1 deficiency in renal collecting ducts leads to PKD and promotes ciliary trafficking\n                      <jats:italic toggle=\"yes\">via<\/jats:italic>\n                      increased KLC3.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1681\/asn.2021111455","type":"journal-article","created":{"date-parts":[[2022,8,12]],"date-time":"2022-08-12T16:25:11Z","timestamp":1660321511000},"page":"1726-1741","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":7,"title":["KLC3 Regulates Ciliary Trafficking and Cyst Progression in CILK1 Deficiency\u2013Related Polycystic Kidney Disease"],"prefix":"10.1681","volume":"33","author":[{"given":"Gyuyeong","family":"Rah","sequence":"first","affiliation":[{"name":"Department of Biological Science, Sookmyung Women\u2019s University, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hwayeon","family":"Cha","sequence":"additional","affiliation":[{"name":"Department of Biological Science, Sookmyung Women\u2019s University, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joohee","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Biological Science, Sookmyung Women\u2019s University, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jieun","family":"Song","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, College of Life Science and Biotechnology, Yonsei University, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hyunho","family":"Kim","sequence":"additional","affiliation":[{"name":"Center for Medical Innovation, Biomedical Research Institute, Seoul National University Hospital, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yun Kyu","family":"Oh","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Seoul National University Boramae Medical Center, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Curie","family":"Ahn","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minyong","family":"Kang","sequence":"additional","affiliation":[{"name":"Department of Urology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jongmin","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Biological Science, Sookmyung Women\u2019s University, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kyung Hyun","family":"Yoo","sequence":"additional","affiliation":[{"name":"Department of Biological Science, Sookmyung Women\u2019s University, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min Jung","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Biological Science, Sookmyung Women\u2019s University, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hyuk Wan","family":"Ko","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, College of Life Science and Biotechnology, Yonsei University, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Je 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