{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,19]],"date-time":"2025-05-19T12:08:57Z","timestamp":1747656537932},"reference-count":24,"publisher":"The Company of Biologists","issue":"Supplement_17","license":[{"start":{"date-parts":[[1993,12,1]],"date-time":"1993-12-01T00:00:00Z","timestamp":754704000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/www.biologists.com\/user-licence-1-1\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1993,12,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Renal cysts are central pathological features in a number of human congenital and acquired diseases, and produce significant morbidity and mortality. This review describes our laboratory\u2019s efforts to identify specific alterations in epithelial cell polarity and differentiation associated with renal tubular cyst formation and progressive enlargement. Studies in a murine model of human autosomal recessive polycystic kidney disease, the C57BL\/6J cpk\/cpk (CPK) mouse have demonstrated quantitative (increased activity) and qualitative (apical membrane distribution) alterations in Na+,K+-adenosine triphosphatase activity that mediate tubular cyst formation. Proximal tubular cyst formation in CPK kidneys is characterized by increased activity of a basolat-eral Na+,K+-ATPase, which drives organic anion secretion and consequent tubular fluid secretion. In contrast, collecting tubule cyst formation is characterized by increased apical membrane Na+,K+-ATPase expression, which may be a marker of the relatively undifferentiated phenotype of cyst lining cells. If such apically expressed enzyme is active, it may have pathogenic import in collecting tubule cyst formation and enlargement by mediating net basal to apical vectorial solute and fluid transport.<\/jats:p>","DOI":"10.1242\/jcs.1993.supplement_17.30","type":"journal-article","created":{"date-parts":[[2013,5,23]],"date-time":"2013-05-23T12:48:12Z","timestamp":1369313292000},"page":"217-222","source":"Crossref","is-referenced-by-count":25,"title":["Epithelial polarity and differentiation in polycystic kidney disease"],"prefix":"10.1242","volume":"1993","author":[{"given":"Ellis D.","family":"Avner","sequence":"first","affiliation":[{"name":"Department of Pediatrics, University of Washington and Division of Nephrology, Children\u2019s Hospital and Medical Center , Seattle, Washington, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"237","published-online":{"date-parts":[[1993,12,1]]},"reference":[{"key":"2024071020073427500_JOCES_1993_S17_217C1","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1159\/000184884","article-title":"Renal cystic disease: insights from recent experimental investigations","volume":"48","author":"Avner","year":"1988","journal-title":"Nephron"},{"key":"2024071020073427500_JOCES_1993_S17_217C2","article-title":"Renal developmental diseases","volume":"13","author":"Avner","year":"1993","journal-title":"Semin. Nephrol"},{"key":"2024071020073427500_JOCES_1993_S17_217C3","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1007\/978-94-009-0457-6_4","article-title":"Models of cysts and cystic kidneys","volume-title":"In The Cystic Kidney","author":"Avner","year":"1990"},{"key":"2024071020073427500_JOCES_1993_S17_217C4","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1007\/BF00853593","article-title":"Congenital murine polycystic kidney disease: I. The ontogeny of tubular cyst formation","volume":"1","author":"Avner","year":"1987","journal-title":"Pediatr. Nephrol"},{"key":"2024071020073427500_JOCES_1993_S17_217C5","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1007\/BF00862522","article-title":"Polypeptide growth factors in metanephric growth and segmental nephron differentiation","volume":"4","author":"Avner","year":"1990","journal-title":"Pediatr. Nephrol"},{"key":"2024071020073427500_JOCES_1993_S17_217C6","doi-asserted-by":"crossref","first-page":"960","DOI":"10.1038\/ki.1989.288","article-title":"In vitro modulation of tubular cyst regression in murine polycystic kidney disease","volume":"36","author":"Avner","year":"1989","journal-title":"Kidney Int"},{"key":"2024071020073427500_JOCES_1993_S17_217C7","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1038\/ki.1985.151","article-title":"Sodium-potassium ATPase activity mediates cyst formation in metanephric organ culture","volume":"28","author":"Avner","year":"1985","journal-title":"Kidney Int"},{"key":"2024071020073427500_JOCES_1993_S17_217C8","doi-asserted-by":"crossref","first-page":"7447","DOI":"10.1073\/pnas.89.16.7447","article-title":"Abnormal sodium pump distribution during renal tubulogenesis in congenital murine polycystic disease","volume":"89","author":"Avner","year":"1992","journal-title":"Proc. Nat. Acad. Sci. USA"},{"key":"2024071020073427500_JOCES_1993_S17_217C9","first-page":"441","article-title":"Triiodothyronine-induced cyst formation in metanephric organ culture: the role of increased Na+,K+-ATPase activity","volume":"109","author":"Avner","year":"1987","journal-title":"J. Lab. Clin. Med"},{"key":"2024071020073427500_JOCES_1993_S17_217C10","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1007\/BF00862593","article-title":"Congenital murine polycystic kidney disease. II. Pathogenesis of tubular cyst formation","volume":"2","author":"Avner","year":"1988","journal-title":"Pediatr. Nephrol"},{"key":"2024071020073427500_JOCES_1993_S17_217C11","doi-asserted-by":"crossref","first-page":"1048","DOI":"10.1681\/ASN.V181048","article-title":"Elevated protooncogene expression in polycystic kidneys of the C57BL\/6J (cpk) mouse","volume":"1","author":"Cowley","year":"1991","journal-title":"J. Amer. Soc. Nephrol"},{"key":"2024071020073427500_JOCES_1993_S17_217C12","first-page":"541","article-title":"A positional cloning approach to inherited renal disease","volume":"12","author":"Germino","year":"1992","journal-title":"Semin. Nephrol"},{"key":"2024071020073427500_JOCES_1993_S17_217C13","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1126\/science.1658934","article-title":"A mechanism for regulating cell surface distribution of Na-K-ATPase in polarized epithelial cells","volume":"254","author":"Hammerton","year":"1991","journal-title":"Science"},{"key":"2024071020073427500_JOCES_1993_S17_217C14","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1016\/0012-1606(91)90249-3","article-title":"The SGP-2 gene is developmentally regulated in the mouse kidney and abnormally expressed in collecting duct cysts in polycystic kidney disease","volume":"146","author":"Harding","year":"1991","journal-title":"Dev. Biol"},{"key":"2024071020073427500_JOCES_1993_S17_217C15","doi-asserted-by":"crossref","first-page":"504","DOI":"10.1203\/00006450-198711000-00005","article-title":"Ontogeny of cell type-specific enzyme reactivities in kidney collecting ducts","volume":"22","author":"Holthofer","year":"1987","journal-title":"Pediatr. Res"},{"key":"2024071020073427500_JOCES_1993_S17_217C16","doi-asserted-by":"crossref","first-page":"1104","DOI":"10.1038\/ki.1987.115","article-title":"Expression of the a-subunit of Na\/K-ATPase in renal collecting duct epithelium during development","volume":"31","author":"Minuth","year":"1987","journal-title":"Kidney Int"},{"key":"2024071020073427500_JOCES_1993_S17_217C17","doi-asserted-by":"crossref","first-page":"556560","DOI":"10.1016\/S0022-5347(17)53911-7","article-title":"Murine congenital polycystic kidney disease: a model for studying development of cystic disease","volume":"127","author":"Preminger","year":"1982","journal-title":"J. Urol"},{"key":"2024071020073427500_JOCES_1993_S17_217C18","first-page":"40","article-title":"Cystic kidneys","volume":"56","author":"Russell","year":"1977","journal-title":"Mouse Newsletter"},{"key":"2024071020073427500_JOCES_1993_S17_217C19","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1007\/BF00768846","article-title":"The Na,K-ATPase","volume":"24","author":"Skou","year":"1992","journal-title":"J. Bioenerg. Biomembr"},{"key":"2024071020073427500_JOCES_1993_S17_217C20","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1007\/BF02388120","article-title":"Intact organ culture of murine metanephros. J","volume":"13","author":"Sweeney","year":"1991","journal-title":"Tiss. Cult. Meth"},{"key":"2024071020073427500_JOCES_1993_S17_217C21","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1007\/978-94-009-0457-6_5","article-title":"Pathogenesis of cysts and cystic kidneys","volume-title":"In The Cystic Kidney","author":"Welling","year":"1990"},{"key":"2024071020073427500_JOCES_1993_S17_217C22","first-page":"1657","article-title":"Cystic and developmental diseases of the kidney","volume-title":"In The Kidney, 4th edn","author":"Welling","year":"1991"},{"key":"2024071020073427500_JOCES_1993_S17_217C23","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1038\/ki.1991.56","article-title":"Tubulocystic epithelium","volume":"39","author":"Wilson","year":"1991","journal-title":"Kidney Int"},{"key":"2024071020073427500_JOCES_1993_S17_217C24","first-page":"Fl","article-title":"Reversed polarity of Na+,K+-ATPase: mislocation to apical plasma membranes in human polycystic kidney disease epithelia","volume":"260","author":"Wilson","year":"1991","journal-title":"Amer. J. Physiol"}],"container-title":["Journal of Cell Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.biologists.com\/jcs\/article-pdf\/1993\/Supplement_17\/217\/3552806\/joces_1993_supplement_17_30.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/journals.biologists.com\/jcs\/article-pdf\/1993\/Supplement_17\/217\/3552806\/joces_1993_supplement_17_30.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,7,10]],"date-time":"2024-07-10T20:08:05Z","timestamp":1720642085000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.biologists.com\/jcs\/article\/1993\/Supplement_17\/217\/58290\/Epithelial-polarity-and-differentiation-in"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1993,12,1]]},"references-count":24,"journal-issue":{"issue":"Supplement_17","published-print":{"date-parts":[[1993,12,1]]}},"URL":"https:\/\/doi.org\/10.1242\/jcs.1993.supplement_17.30","relation":{},"ISSN":["0021-9533","1477-9137"],"issn-type":[{"value":"0021-9533","type":"print"},{"value":"1477-9137","type":"electronic"}],"subject":[],"published":{"date-parts":[[1993,12,1]]}}}