{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T17:28:03Z","timestamp":1788456483117,"version":"build-2803163510"},"reference-count":68,"publisher":"Oxford University Press (OUP)","issue":"18","license":[{"start":{"date-parts":[[2020,9,4]],"date-time":"2020-09-04T00:00:00Z","timestamp":1599177600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["DK099434"],"award-info":[{"award-number":["DK099434"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["P30DK079307"],"award-info":[{"award-number":["P30DK079307"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["DK115403"],"award-info":[{"award-number":["DK115403"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["DK068306"],"award-info":[{"award-number":["DK068306"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100013368","name":"German National Academy of Sciences Leopoldina","doi-asserted-by":"publisher","award":["LPDS-2015-07"],"award-info":[{"award-number":["LPDS-2015-07"]}],"id":[{"id":"10.13039\/501100013368","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"German Research Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100010782","name":"Cluster of Excellence Inflammation at Interfaces","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100010782","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005624","name":"Hannover Medical School","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005624","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,11,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>ANKS6 is a ciliary protein that localizes to the proximal compartment of the primary cilium, where it regulates signaling. Mutations in the ANKS6 gene cause multiorgan ciliopathies in humans, which include laterality defects of the visceral organs, renal cysts as part of nephronophthisis and congenital hepatic fibrosis (CHF) in the liver. Although CHF together with liver ductal plate malformations are common features of several human ciliopathy syndromes, including nephronophthisis-related ciliopathies, the mechanism by which mutations in ciliary genes lead to bile duct developmental abnormalities is not understood. Here, we generated a knockout mouse model of Anks6 and show that ANKS6 function is required for bile duct morphogenesis and cholangiocyte differentiation. The loss of Anks6 causes ciliary abnormalities, ductal plate remodeling defects and periportal fibrosis in the liver. Our expression studies and biochemical analyses show that biliary abnormalities in Anks6-deficient livers result from the dysregulation of YAP transcriptional activity in the bile duct-lining epithelial cells. Mechanistically, our studies suggest, that ANKS6 antagonizes Hippo signaling in the liver during bile duct development by binding to Hippo pathway effector proteins YAP1, TAZ and TEAD4 and promoting their transcriptional activity. Together, this study reveals a novel function for ANKS6 in regulating Hippo signaling during organogenesis and provides mechanistic insights into the regulatory network controlling bile duct differentiation and morphogenesis during liver development.<\/jats:p>","DOI":"10.1093\/hmg\/ddaa197","type":"journal-article","created":{"date-parts":[[2020,9,1]],"date-time":"2020-09-01T11:10:28Z","timestamp":1598958628000},"page":"3064-3080","source":"Crossref","is-referenced-by-count":21,"title":["Loss of<i>Anks6<\/i>leads to YAP deficiency and liver abnormalities"],"prefix":"10.1093","volume":"29","author":[{"given":"Merlin","family":"Airik","sequence":"first","affiliation":[{"name":"Division of Nephrology, Department of Pediatrics, University of Pittsburgh, Pittsburgh, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Sch\u00fcler","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Internal Intensive Care Medicine, Charite University, Berlin, Germany"},{"name":"Division of Nephrology, Boston Children\u2019s Hospital, Boston, MA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Blake","family":"McCourt","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Pediatrics, University of Pittsburgh, Pittsburgh, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna-Carina","family":"Weiss","sequence":"additional","affiliation":[{"name":"Department for Molecular Biology, Hannover, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathan","family":"Herdman","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Pediatrics, University of Pittsburgh, Pittsburgh, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timo H","family":"L\u00fcdtke","sequence":"additional","affiliation":[{"name":"Department for Molecular Biology, Hannover, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7773-5190","authenticated-orcid":false,"given":"Eugen","family":"Widmeier","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Boston Children\u2019s Hospital, Boston, MA, USA"},{"name":"Department of Medicine, Renal Division, Medical Center - University of Freiburg, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donna B","family":"Stolz","sequence":"additional","affiliation":[{"name":"Department of Cell Biology, University of Pittsburgh, Pittsburgh, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kari N","family":"Nejak-Bowen","sequence":"additional","affiliation":[{"name":"Division of Experimental Pathology, Department of Pathology, University of Pittsburgh, Pittsburgh, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dean","family":"Yimlamai","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology and Nutrition, Department of Pediatrics, University of Pittsburgh, Pittsburgh, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yijen L","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Developmental Biology, University of Pittsburgh, Pittsburgh, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Kispert","sequence":"additional","affiliation":[{"name":"Department for Molecular Biology, Hannover, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7055-6325","authenticated-orcid":false,"given":"Rannar","family":"Airik","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Pediatrics, University of Pittsburgh, Pittsburgh, PA, USA"},{"name":"Department of Developmental Biology, University of Pittsburgh, Pittsburgh, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Friedhelm","family":"Hildebrandt","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Boston Children\u2019s Hospital, Boston, MA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,9,4]]},"reference":[{"key":"2020121214133474700_ref1","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. 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Genet."},{"key":"2020121214133474700_ref5","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1038\/ejhg.2012.115","article-title":"Bardet-Biedl syndrome","volume":"21","author":"Forsythe","year":"2013","journal-title":"Eur. J. Hum. Genet."},{"key":"2020121214133474700_ref6","doi-asserted-by":"crossref","first-page":"1533","DOI":"10.1056\/NEJMra1010172","article-title":"Ciliopathies","volume":"364","author":"Hildebrandt","year":"2011","journal-title":"N. Engl. J. Med."},{"key":"2020121214133474700_ref7","doi-asserted-by":"crossref","first-page":"2640","DOI":"10.1002\/ajmg.a.33666","article-title":"Fibrocystic disease of liver and pancreas; under-recognized features of the X-linked ciliopathy oral-facial-digital syndrome type 1 (OFD I)","volume":"152A","author":"Chetty-John","year":"2010","journal-title":"Am. J. Med. Genet. 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Genet."},{"key":"2020121214133474700_ref25","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1086\/505332","article-title":"NOTCH2 mutations cause Alagille syndrome, a heterogeneous disorder of the NOTCH signaling pathway","volume":"79","author":"McDaniell","year":"2006","journal-title":"Am. J. Hum. Genet."},{"key":"2020121214133474700_ref26","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1038\/ng0797-235","article-title":"Mutations in the human Jagged1 gene are responsible for Alagille syndrome","volume":"16","author":"Oda","year":"1997","journal-title":"Nat. Genet."},{"key":"2020121214133474700_ref27","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1101\/gad.210773.112","article-title":"The hippo pathway: regulators and regulations","volume":"27","author":"Yu","year":"2013","journal-title":"Genes Dev."},{"key":"2020121214133474700_ref28","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1016\/j.cell.2015.10.044","article-title":"Hippo pathway in organ size control, tissue homeostasis, and cancer","volume":"163","author":"Yu","year":"2015","journal-title":"Cell"},{"key":"2020121214133474700_ref29","doi-asserted-by":"crossref","first-page":"1229","DOI":"10.1101\/gad.888601","article-title":"TEF transcription factors utilize the activation domain of YAP65, a Src\/Yes-associated protein localized in the cytoplasm","volume":"15","author":"Vassilev","year":"2001","journal-title":"Genes Dev."},{"key":"2020121214133474700_ref30","doi-asserted-by":"crossref","first-page":"4059","DOI":"10.1242\/dev.027151","article-title":"Mammalian Tead proteins regulate cell proliferation and contact inhibition as transcriptional mediators of Hippo signaling","volume":"135","author":"Ota","year":"2008","journal-title":"Development"},{"key":"2020121214133474700_ref31","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1038\/ncb3084","article-title":"Dual role of YAP and TAZ in renewal of the intestinal epithelium","volume":"17","author":"Imajo","year":"2015","journal-title":"Nat. Cell Biol."},{"key":"2020121214133474700_ref32","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1016\/j.devcel.2014.06.019","article-title":"Notch and hippo converge on Cdx2 to specify the trophectoderm lineage in the mouse blastocyst","volume":"30","author":"Rayon","year":"2014","journal-title":"Dev. Cell"},{"key":"2020121214133474700_ref33","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.devcel.2010.06.015","article-title":"The Merlin\/NF2 tumor suppressor functions through the YAP oncoprotein to regulate tissue homeostasis in mammals","volume":"19","author":"Zhang","year":"2010","journal-title":"Dev. Cell"},{"key":"2020121214133474700_ref34","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":"2020121214133474700_ref35","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1038\/ng1216","article-title":"Mutations in a novel gene, NPHP3, cause adolescent nephronophthisis, tapeto-retinal degeneration and hepatic fibrosis","volume":"34","author":"Olbrich","year":"2003","journal-title":"Nat. Genet."},{"key":"2020121214133474700_ref36","doi-asserted-by":"crossref","first-page":"877","DOI":"10.1007\/s00431-011-1552-0","article-title":"Caroli disease, bilateral diffuse cystic renal dysplasia, situs inversus, postaxial polydactyly, and preauricular fistulas: a ciliopathy caused by a homozygous NPHP3 mutation","volume":"172","author":"Calinescu-Tuleasca","year":"2013","journal-title":"Eur. J. Pediatr."},{"key":"2020121214133474700_ref37","doi-asserted-by":"crossref","first-page":"951","DOI":"10.1038\/ng.2681","article-title":"ANKS6 is a central component of a nephronophthisis module linking NEK8 to INVS and NPHP3","volume":"45","author":"Hoff","year":"2013","journal-title":"Nat. Genet."},{"key":"2020121214133474700_ref38","doi-asserted-by":"crossref","first-page":"5528","DOI":"10.1093\/hmg\/dds408","article-title":"The ciliopathy disease protein NPHP9 promotes nuclear delivery and activation of the oncogenic transcriptional regulator TAZ","volume":"21","author":"Habbig","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"2020121214133474700_ref39","doi-asserted-by":"crossref","first-page":"2177","DOI":"10.1093\/hmg\/ddt070","article-title":"Mutations in NEK8 link multiple organ dysplasia with altered hippo signalling and increased c-MYC expression","volume":"22","author":"Frank","year":"2013","journal-title":"Hum. Mol. 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Commun."},{"key":"2020121214133474700_ref43","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1038\/ki.2015.122","article-title":"The SAM domain of ANKS6 has different interacting partners and mutations can induce different cystic phenotypes","volume":"88","author":"Bakey","year":"2015","journal-title":"Kidney Int."},{"key":"2020121214133474700_ref44","doi-asserted-by":"crossref","first-page":"3517","DOI":"10.1681\/ASN.2005060601","article-title":"Missense mutation in sterile alpha motif of novel protein SamCystin is associated with polycystic kidney disease in (cy\/+) rat","volume":"16","author":"Brown","year":"2005","journal-title":"J. Am. Soc. Nephrol."},{"key":"2020121214133474700_ref45","doi-asserted-by":"crossref","first-page":"3000","DOI":"10.2353\/ajpath.2010.100569","article-title":"Transgenic overexpression of Anks6(p.R823W) causes polycystic kidney disease in rats","volume":"177","author":"Neudecker","year":"2010","journal-title":"Am. J. 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