{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T05:45:42Z","timestamp":1792820742793,"version":"4.5.0"},"reference-count":31,"publisher":"Elsevier BV","issue":"5","license":[{"start":{"date-parts":[[2003,11,1]],"date-time":"2003-11-01T00:00:00Z","timestamp":1067644800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Gastroenterology"],"published-print":{"date-parts":[[2003,11]]},"DOI":"10.1016\/j.gastro.2003.09.001","type":"journal-article","created":{"date-parts":[[2003,11,3]],"date-time":"2003-11-03T12:23:16Z","timestamp":1067862196000},"page":"1303-1310","source":"Crossref","is-referenced-by-count":186,"title":["Defects in cholangiocyte fibrocystin expression and ciliary structure in the PCK rat1 1The authors thank Dr. Torra for supplying ARPKD tissue."],"prefix":"10.1016","volume":"125","author":[{"given":"Tatyana V","family":"Masyuk","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bing Q","family":"Huang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher J","family":"Ward","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anatoliy I","family":"Masyuk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Yuan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick L","family":"Splinter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachaneekorn","family":"Punyashthiti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric L","family":"Ritman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vicente E","family":"Torres","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter C","family":"Harris","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas F","family":"Larusso","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.gastro.2003.09.001_BIB1","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1086\/368295","article-title":"Mutations in PRKCSH cause isolated autosomal dominant polycystic liver disease","volume":"72","author":"Li","year":"2003","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.gastro.2003.09.001_BIB2","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1053\/snep.2001.20929","article-title":"The genetics and physiology of polycystic kidney disease","volume":"21","author":"Calvet","year":"2001","journal-title":"Semin Nephrol"},{"key":"10.1016\/j.gastro.2003.09.001_BIB3","series-title":"Polycystic kidney disease","first-page":"237","article-title":"Autosomal recessive polycystic kidney disease","author":"Guay-Woodford","year":"1996"},{"key":"10.1016\/j.gastro.2003.09.001_BIB4","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1002\/(SICI)1096-8628(19980305)76:2<137::AID-AJMG6>3.0.CO;2-Q","article-title":"Prenatal diagnosis of autosomal recessive polycystic kidney disease (ARPKD)","volume":"76","author":"Zerres","year":"1998","journal-title":"Am J Med Genet"},{"key":"10.1016\/j.gastro.2003.09.001_BIB5","doi-asserted-by":"crossref","first-page":"1605","DOI":"10.1016\/S0002-9440(10)64116-8","article-title":"Polycystic kidney rat is a novel animal model of Caroli\u2019s disease associated with congenital hepatic fibrosis","volume":"158","author":"Sanzen","year":"2001","journal-title":"Am J Pathol"},{"key":"10.1016\/j.gastro.2003.09.001_BIB6","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1097\/01.ASN.0000039578.55705.6E","article-title":"Spectrum of mutations in the gene for autosomal recessive polycystic kidney disease (ARPKD\/PKHD1)","volume":"14","author":"Bergmann","year":"2003","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.gastro.2003.09.001_BIB7","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1038\/ng833","article-title":"The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein","volume":"30","author":"Ward","year":"2002","journal-title":"Nat Genet"},{"key":"10.1016\/j.gastro.2003.09.001_BIB8","doi-asserted-by":"crossref","first-page":"1305","DOI":"10.1086\/340448","article-title":"PKHD1, the polycystic kidney and hepatic disease 1 gene, encodes a novel large protein containing multiple immunoglobulin-like plexin-transcription-factor domains and parallel beta-helix 1 repeats","volume":"70","author":"Onuchic","year":"2002","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.gastro.2003.09.001_BIB9","first-page":"F541","article-title":"Polaris, a protein disrupted in orpk mutant mice, is required for assembly of renal cilium","volume":"282","author":"Yoder","year":"2002","journal-title":"Am J Physiol"},{"key":"10.1016\/j.gastro.2003.09.001_BIB10","doi-asserted-by":"crossref","first-page":"709","DOI":"10.1083\/jcb.151.3.709","article-title":"Chlamydomonas IFT88 and its mouse homologue, polycystic kidney disease gene tg737, are required for assembly of cilia and flagella","volume":"151","author":"Pazour","year":"2000","journal-title":"J Cell Biol"},{"key":"10.1016\/j.gastro.2003.09.001_BIB11","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1091\/mbc.12.3.589","article-title":"Polaris, a protein involved in left-right axis patterning, localizes to basal bodies and cilia","volume":"12","author":"Taulman","year":"2001","journal-title":"Mol Biol Cell"},{"key":"10.1016\/j.gastro.2003.09.001_BIB12","doi-asserted-by":"crossref","first-page":"2347","DOI":"10.1242\/dev.127.11.2347","article-title":"The Oak Ridge Polycystic Kidney (ORPK) disease gene is required for left-right axis determination","volume":"127","author":"Murcia","year":"2000","journal-title":"Development"},{"key":"10.1016\/j.gastro.2003.09.001_BIB13","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1083\/jcb.200107108","article-title":"The intraflagellar protein, IFT88, is essential for vertebrate ptotoreceptor assembly and maintenance","volume":"157","author":"Pazour","year":"2002","journal-title":"J Cell Biol"},{"key":"10.1016\/j.gastro.2003.09.001_BIB14","doi-asserted-by":"crossref","first-page":"1493","DOI":"10.1242\/dev.128.9.1493","article-title":"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","volume":"128","author":"Haycraft","year":"2001","journal-title":"Development"},{"key":"10.1016\/j.gastro.2003.09.001_BIB15","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1016\/S0960-9822(01)00122-1","article-title":"An autosomal recessive polycystic kidney disease gene homologue is involved in intraflagellar transport in C. elegans ciliated sensory neurons","volume":"11","author":"Qin","year":"2001","journal-title":"Curr Biol"},{"key":"10.1016\/j.gastro.2003.09.001_BIB16","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1038\/nrm952","article-title":"Intraflagellar transport","volume":"3","author":"Rosenbaum","year":"2002","journal-title":"Nat Rev Mol Cell Biol"},{"key":"10.1016\/j.gastro.2003.09.001_BIB17","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1172\/JCI0214099","article-title":"Cystin, a novel cilia-associated protein, is disrupted in the cpk mouse model of polycystic kidney disease","volume":"109","author":"Hou","year":"2002","journal-title":"J Clin Invest"},{"key":"10.1016\/j.gastro.2003.09.001_BIB18","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1038\/ng1217","article-title":"Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination","volume":"34","author":"Otto","year":"2003","journal-title":"Nat Genet"},{"key":"10.1016\/j.gastro.2003.09.001_BIB19","doi-asserted-by":"crossref","first-page":"2508","DOI":"10.1097\/01.ASN.0000029587.47950.25","article-title":"The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia","volume":"13","author":"Yoder","year":"2002","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.gastro.2003.09.001_BIB20","doi-asserted-by":"crossref","first-page":"2614","DOI":"10.1681\/ASN.V13102614","article-title":"Cilia in PKD\u2014letting it all hang out","volume":"13","author":"Calvet","year":"2002","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.gastro.2003.09.001_BIB21","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1016\/S0140-6736(03)12662-1","article-title":"Polycystic kidney disease\u2014the ciliary connection","volume":"361","author":"Ong","year":"2003","journal-title":"Lancet"},{"key":"10.1016\/j.gastro.2003.09.001_BIB22","doi-asserted-by":"crossref","first-page":"2079","DOI":"10.1016\/S0002-9440(10)64679-2","article-title":"Quantitative assessment of the rat intrahepatic biliary system by three-dimensional reconstruction","volume":"158","author":"Masyuk","year":"2001","journal-title":"Am J Pathol"},{"key":"10.1016\/j.gastro.2003.09.001_BIB23","doi-asserted-by":"crossref","DOI":"10.1093\/hmg\/ddg274","article-title":"Cellular and subcellular localization of the ARPKD protein; fibrocystin is expressed on primary cilia","author":"Ward","year":"2003","journal-title":"Human Mol Genet"},{"key":"10.1016\/j.gastro.2003.09.001_BIB24","doi-asserted-by":"crossref","first-page":"28557","DOI":"10.1074\/jbc.274.40.28557","article-title":"Identification and characterization of polycystin-2, the PKD2 gene product","volume":"274","author":"Cai","year":"1999","journal-title":"J Biol Chem"},{"key":"10.1016\/j.gastro.2003.09.001_BIB25","doi-asserted-by":"crossref","first-page":"2246","DOI":"10.1097\/01.ASN.0000030392.19694.9D","article-title":"Identification and characterization of Pkhd1, the mouse orthologue of the human ARPKD gene","volume":"13","author":"Nagasawa","year":"2002","journal-title":"J Am Soc Nephrol"},{"key":"10.1016\/j.gastro.2003.09.001_BIB26","doi-asserted-by":"crossref","first-page":"9080","DOI":"10.1073\/pnas.90.19.9080","article-title":"Regulation of bicarbonate-dependent ductular bile secretion assessed by lumenal micropuncture of isolated rodent intrahepatic bile ducts","volume":"90","author":"Roberts","year":"1993","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.gastro.2003.09.001_BIB27","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1006\/cbir.1996.0011","article-title":"Expression of primary cilia in mammalian cells","volume":"20","author":"Wheatley","year":"1996","journal-title":"Cell Biol Int"},{"key":"10.1016\/j.gastro.2003.09.001_BIB28","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1159\/000163955","article-title":"Primary cilia in normal and pathological tissues","volume":"63","author":"Wheatley","year":"1995","journal-title":"Pathobiology"},{"key":"10.1016\/j.gastro.2003.09.001_BIB29","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1002\/aja.1001440304","article-title":"Scanning electron microscopy of normal rat liver","volume":"144","author":"Grisham","year":"1975","journal-title":"Am J Anat"},{"key":"10.1016\/j.gastro.2003.09.001_BIB30","doi-asserted-by":"crossref","first-page":"6268","DOI":"10.1074\/jbc.M212079200","article-title":"Specific inhibition of AQP1 water channels in isolated rat intrahepatic bile duct units by small interfering RNAs","volume":"278","author":"Splinter","year":"2003","journal-title":"J Biol Chem"},{"key":"10.1016\/j.gastro.2003.09.001_BIB31","doi-asserted-by":"crossref","first-page":"5286","DOI":"10.1073\/pnas.0836980100","article-title":"Kidney-specific inactivation of the KIF3A subunit of kinesis-II inhibits renal ciliogenesis and produces polycystic kidney disease","volume":"100","author":"Lin","year":"2003","journal-title":"Proc Natl Acad Sci U S A"}],"container-title":["Gastroenterology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0016508503013696?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0016508503013696?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2021,6,12]],"date-time":"2021-06-12T11:36:51Z","timestamp":1623497811000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0016508503013696"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2003,11]]},"references-count":31,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2003,11]]}},"alternative-id":["S0016508503013696"],"URL":"https:\/\/doi.org\/10.1016\/j.gastro.2003.09.001","relation":{},"ISSN":["0016-5085"],"issn-type":[{"value":"0016-5085","type":"print"}],"subject":[],"published":{"date-parts":[[2003,11]]}}}