{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T00:35:23Z","timestamp":1791333323376,"version":"4.3.1"},"reference-count":41,"publisher":"Elsevier BV","issue":"9","license":[{"start":{"date-parts":[[2011,5,1]],"date-time":"2011-05-01T00:00:00Z","timestamp":1304208000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2015,12,7]],"date-time":"2015-12-07T00:00:00Z","timestamp":1449446400000},"content-version":"vor","delay-in-days":1681,"URL":"https:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Kidney International"],"published-print":{"date-parts":[[2011,5]]},"DOI":"10.1038\/ki.2010.538","type":"journal-article","created":{"date-parts":[[2011,1,26]],"date-time":"2011-01-26T14:16:54Z","timestamp":1296051414000},"page":"1013-1025","source":"Crossref","is-referenced-by-count":55,"title":["Bardet\u2013Biedl syndrome highlights the major role of the primary cilium in efficient water reabsorption"],"prefix":"10.1016","volume":"79","author":[{"given":"Vincent","family":"Marion","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique","family":"Schlicht","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana\u00efs","family":"Mockel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Caillard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Imhoff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corinne","family":"Stoetzel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"van Dijk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Brandt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno","family":"Moulin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00e9l\u00e8ne","family":"Dollfus","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1038\/ki.2010.538_bb0010","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1002\/ajmg.c.30225","article-title":"Liver and kidney disease in ciliopathies","volume":"151C","author":"Gunay-Aygun","year":"2009","journal-title":"Am J Med Genet C Semin Med Genet"},{"key":"10.1038\/ki.2010.538_bb0015","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1056\/NEJM198809083191005","article-title":"The spectrum of renal disease in Laurence-Moon-Biedl syndrome","volume":"319","author":"Harnett","year":"1988","journal-title":"N Engl J Med"},{"key":"10.1038\/ki.2010.538_bb0020","doi-asserted-by":"crossref","first-page":"776","DOI":"10.1016\/S0272-6386(96)90513-2","article-title":"The importance of renal impairment in the natural history of Bardet-Biedl syndrome","volume":"27","author":"O\u2019Dea","year":"1996","journal-title":"Am J Kidney Dis"},{"key":"10.1038\/ki.2010.538_bb0025","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1172\/JCI37041","article-title":"Mechanistic insights into Bardet-Biedl syndrome, a model ciliopathy","volume":"119","author":"Zaghloul","year":"2009","journal-title":"J Clin Invest"},{"key":"10.1038\/ki.2010.538_bb0030","doi-asserted-by":"crossref","first-page":"1201","DOI":"10.1016\/j.cell.2007.03.053","article-title":"A core complex of BBS proteins cooperates with the GTPase Rab8 to promote ciliary membrane biogenesis","volume":"129","author":"Nachury","year":"2007","journal-title":"Cell"},{"key":"10.1038\/ki.2010.538_bb0035","doi-asserted-by":"crossref","first-page":"1488","DOI":"10.1073\/pnas.0910268107","article-title":"BBS6, BBS10, and BBS12 form a complex with CCT\/TRiC family chaperonins and mediate BBSome assembly","volume":"107","author":"Seo","year":"2010","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1038\/ki.2010.538_bb0040","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1136\/jmg.36.6.437","article-title":"New criteria for improved diagnosis of Bardet-Biedl syndrome: results of a population survey","volume":"36","author":"Beales","year":"1999","journal-title":"J Med Genet"},{"key":"10.1038\/ki.2010.538_bb0045","doi-asserted-by":"crossref","first-page":"1925","DOI":"10.1046\/j.1523-1755.2002.00305.x","article-title":"Clinical and genetic epidemiology of inherited renal disease in Newfoundland","volume":"61","author":"Parfrey","year":"2002","journal-title":"Kidney Int"},{"key":"10.1038\/ki.2010.538_bb0050","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1111\/j.1651-2227.1986.tb10192.x","article-title":"Renal involvement in the Laurence-Moon-Biedl syndrome. Functional and radiological studies","volume":"75","author":"Linne","year":"1986","journal-title":"Acta Paediatr Scand"},{"key":"10.1038\/ki.2010.538_bb0055","doi-asserted-by":"crossref","first-page":"16588","DOI":"10.1073\/pnas.0405496101","article-title":"Bbs2-null mice have neurosensory deficits, a defect in social dominance, and retinopathy associated with mislocalization of rhodopsin","volume":"101","author":"Nishimura","year":"2004","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1038\/ki.2010.538_bb0060","doi-asserted-by":"crossref","first-page":"1288","DOI":"10.2353\/ajpath.2006.050941","article-title":"ADP-ribosylation factor-like 3 is involved in kidney and photoreceptor development","volume":"168","author":"Schrick","year":"2006","journal-title":"Am J Pathol"},{"key":"10.1038\/ki.2010.538_bb0065","doi-asserted-by":"crossref","first-page":"8664","DOI":"10.1073\/pnas.0402354101","article-title":"Bardet-Biedl syndrome type 4 (BBS4)-null mice implicate Bbs4 in flagella formation but not global cilia assembly","volume":"101","author":"Mykytyn","year":"2004","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1038\/ki.2010.538_bb0070","doi-asserted-by":"crossref","first-page":"1109","DOI":"10.1093\/hmg\/ddi123","article-title":"Mkks-null mice have a phenotype resembling Bardet-Biedl syndrome","volume":"14","author":"Fath","year":"2005","journal-title":"Hum Mol Genet"},{"key":"10.1038\/ki.2010.538_bb0075","first-page":"199","article-title":"Renal abnormalities in the Bardet-Biedl syndrome","volume":"3","author":"Tieder","year":"1982","journal-title":"Int J Pediatr Nephrol"},{"key":"10.1038\/ki.2010.538_bb0080","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1007\/BF00857673","article-title":"Early diagnosis of Bardet-Biedl syndrome","volume":"4","author":"Fralick","year":"1990","journal-title":"Pediatr Nephrol"},{"key":"10.1038\/ki.2010.538_bb0085","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1007\/s004670050228","article-title":"Renal involvement in the Laurence-Moon-Bardet-Biedl syndrome: report of five cases","volume":"11","author":"Ucar","year":"1997","journal-title":"Pediatr Nephrol"},{"key":"10.1038\/ki.2010.538_bb0090","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1097\/00041552-199801000-00007","article-title":"Physiology and pathophysiology of the aquaporin-2 water channel","volume":"7","author":"Deen","year":"1998","journal-title":"Curr Opin Nephrol Hypertens"},{"key":"10.1038\/ki.2010.538_bb0095","first-page":"F254","article-title":"Arginine vasopressin stimulates phosphorylation of aquaporin-2 in rat renal tissue","volume":"276","author":"Nishimoto","year":"1999","journal-title":"Am J Physiol"},{"key":"10.1038\/ki.2010.538_bb0100","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1038\/nrneph.2009.231","article-title":"Aquaporins in kidney pathophysiology","volume":"6","author":"Noda","year":"2010","journal-title":"Nat Rev Nephrol"},{"key":"10.1038\/ki.2010.538_bb0105","doi-asserted-by":"crossref","first-page":"1768","DOI":"10.1172\/JCI118222","article-title":"Immunohistochemical localization of V2 vasopressin receptor along the nephron and functional role of luminal V2 receptor in terminal inner medullary collecting ducts","volume":"96","author":"Nonoguchi","year":"1995","journal-title":"J Clin Invest"},{"key":"10.1038\/ki.2010.538_bb0110","doi-asserted-by":"crossref","first-page":"F87","DOI":"10.1152\/ajprenal.90509.2008","article-title":"Vasopressin receptor-mediated functional signaling pathway in primary cilia of renal epithelial cells","volume":"296","author":"Raychowdhury","year":"2009","journal-title":"Am J Physiol Renal Physiol"},{"key":"10.1038\/ki.2010.538_bb0115","first-page":"F20","article-title":"Electrophysiological study of luminal and basolateral vasopressin in rabbit cortical collecting duct","volume":"268","author":"Naruse","year":"1995","journal-title":"Am J Physiol"},{"key":"10.1038\/ki.2010.538_bb0120","article-title":"Bardet-Biedl Syndrome: A Study of the Renal and Cardiovascular Phenotypes in a French Cohort","author":"Imhoff","year":"2010","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1038\/ki.2010.538_bb0125","doi-asserted-by":"crossref","first-page":"3781","DOI":"10.1210\/jc.2008-2508","article-title":"Body mass index is associated with increased creatinine clearance by a mechanism independent of body fat distribution","volume":"94","author":"Gerchman","year":"2009","journal-title":"J Clin Endocrinol Metab"},{"key":"10.1038\/ki.2010.538_bb0130","doi-asserted-by":"crossref","first-page":"F817","DOI":"10.1152\/ajprenal.2000.278.5.F817","article-title":"Glomerular hemodynamics in severe obesity","volume":"278","author":"Chagnac","year":"2000","journal-title":"Am J Physiol Renal Physiol"},{"key":"10.1038\/ki.2010.538_bb0135","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1053\/j.ajkd.2010.06.005","article-title":"Obesity, glomerular hyperfiltration, and the surface area correction","volume":"56","author":"Levey","year":"2010","journal-title":"Am J Kidney Dis"},{"key":"10.1038\/ki.2010.538_bb0140","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1038\/ng1771","article-title":"BBS10 encodes a vertebrate-specific chaperonin-like protein and is a major BBS locus","volume":"38","author":"Stoetzel","year":"2006","journal-title":"Nat Genet"},{"key":"10.1038\/ki.2010.538_bb0145","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1086\/510256","article-title":"Identification of a novel BBS gene (BBS12) highlights the major role of a vertebrate-specific branch of chaperonin-related proteins in Bardet-Biedl syndrome","volume":"80","author":"Stoetzel","year":"2007","journal-title":"Am J Hum Genet"},{"key":"10.1038\/ki.2010.538_bb0150","doi-asserted-by":"crossref","first-page":"1007","DOI":"10.1242\/jcs.01676","article-title":"MKKS\/BBS6, a divergent chaperonin-like protein linked to the obesity disorder Bardet-Biedl syndrome, is a novel centrosomal component required for cytokinesis","volume":"118","author":"Kim","year":"2005","journal-title":"J Cell Sci"},{"key":"10.1038\/ki.2010.538_bb0155","doi-asserted-by":"crossref","first-page":"F87","DOI":"10.1152\/ajprenal.90509.2008","article-title":"Vasopressin receptor-mediated functional signaling pathway in primary cilia of renal epithelial cells","volume":"296","author":"Raychowdhury","year":"2009","journal-title":"Am J Physiol Renal Physiol"},{"key":"10.1038\/ki.2010.538_bb0160","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1152\/physrev.00024.2001","article-title":"Aquaporins in the kidney: from molecules to medicine","volume":"82","author":"Nielsen","year":"2002","journal-title":"Physiol Rev"},{"key":"10.1038\/ki.2010.538_bb0165","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/j.jtbi.2008.10.031","article-title":"Cell volume distributions reveal cell growth rates and division times","volume":"257","author":"Halter","year":"2009","journal-title":"J Theor Biol"},{"key":"10.1038\/ki.2010.538_bb0170","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1126\/science.1174294","article-title":"Cell growth and size homeostasis in proliferating animal cells","volume":"325","author":"Tzur","year":"2009","journal-title":"Science"},{"key":"10.1038\/ki.2010.538_bb0175","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1681\/ASN.2008050456","article-title":"Nephronophthisis: disease mechanisms of a ciliopathy","volume":"20","author":"Hildebrandt","year":"2009","journal-title":"J Am Soc Nephrol"},{"key":"10.1038\/ki.2010.538_bb0180","doi-asserted-by":"crossref","first-page":"1002","DOI":"10.1056\/NEJM198910123211503","article-title":"The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome","volume":"321","author":"Green","year":"1989","journal-title":"N Engl J Med"},{"key":"10.1038\/ki.2010.538_bb0185","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1002\/ajmg.a.30406","article-title":"Clinical and genetic epidemiology of Bardet-Biedl syndrome in Newfoundland: a 22-year prospective, population-based, cohort study","volume":"132","author":"Moore","year":"2005","journal-title":"Am J Med Genet A"},{"key":"10.1038\/ki.2010.538_bb0190","doi-asserted-by":"crossref","first-page":"1740","DOI":"10.1681\/ASN.2006010052","article-title":"PKD1 haploinsufficiency causes a syndrome of inappropriate antidiuresis in mice","volume":"18","author":"Ahrabi","year":"2007","journal-title":"J Am Soc Nephrol"},{"key":"10.1038\/ki.2010.538_bb0195","first-page":"R365","article-title":"Urinary and metabolic clearances of arginine vasopressin in normal subjects","volume":"251","author":"Moses","year":"1986","journal-title":"Am J Physiol"},{"key":"10.1038\/ki.2010.538_bb0200","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1146\/annurev.ph.56.030194.003431","article-title":"Hormonal signaling and regulation of salt and water transport in the collecting duct","volume":"56","author":"Breyer","year":"1994","journal-title":"Annu Rev Physiol"},{"key":"10.1038\/ki.2010.538_bb0205","doi-asserted-by":"crossref","first-page":"1310","DOI":"10.1038\/ki.1995.186","article-title":"Role of adenosine on glucagon-induced cAMP in a human cortical collecting duct cell line","volume":"47","author":"Prie","year":"1995","journal-title":"Kidney Int"},{"key":"10.1038\/ki.2010.538_bb0210","doi-asserted-by":"crossref","first-page":"e45","DOI":"10.1093\/nar\/gkp045","article-title":"Amplification efficiency: linking baseline and bias in the analysis of quantitative PCR data","volume":"37","author":"Ruijter","year":"2009","journal-title":"Nucleic Acids Res"}],"container-title":["Kidney International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0085253815549132?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0085253815549132?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2023,6,5]],"date-time":"2023-06-05T00:03:01Z","timestamp":1685923381000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0085253815549132"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,5]]},"references-count":41,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2011,5]]}},"alternative-id":["S0085253815549132"],"URL":"https:\/\/doi.org\/10.1038\/ki.2010.538","relation":{},"ISSN":["0085-2538"],"issn-type":[{"value":"0085-2538","type":"print"}],"subject":[],"published":{"date-parts":[[2011,5]]}}}