{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,25]],"date-time":"2026-02-25T08:35:53Z","timestamp":1772008553419,"version":"3.50.1"},"reference-count":41,"publisher":"American Physiological Society","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American Journal of Physiology-Renal Physiology"],"published-print":{"date-parts":[[2004,2]]},"abstract":"<jats:p>The main feature of polycystic kidney diseases (PKD) is formation and progressive enlargement of renal cysts. Alterations in epithelial cell proliferation, extracellular matrix, and ion transport are thought to contribute to cyst enlargement and loss of renal function. Abnormal Cl<jats:sup>-<\/jats:sup>secretion is implicated in cyst enlargement in autosomal dominant PKD (ADPKD), but little is known about transport abnormalities in autosomal recessive PKD (ARPKD). We developed a method to isolate collecting duct (CD) principal cells (site of the lesion in ARPKD) from normal and ARPKD mice. A transgenic mouse (Hoxb7\/GFP) in which enhanced green fluorescent protein (GFP) is expressed in CDs was bred with an ARPKD mouse (BPK), and GFP-positive cells from normal and cystic mice were selected by fluorescence-activated cell sorting. GFP-positive CD cells (&gt;95 \u00b1 3%) obtained from either normal or cystic mice formed high-resistance, polarized epithelial monolayers. Expression patterns for marker proteins and the presence of a central cilium confirmed that the monolayers are composed of principal cells. Under basal conditions, the Cl<jats:sup>-<\/jats:sup>secretory responses elicited by elevation of cAMP or calcium were not significantly different between normal and cystic monolayers. In contrast, the amiloride-sensitive short-circuit current was significantly reduced in monolayers of cells isolated from cystic mice (12.9 \u00b1 1.6 \u03bcA\/cm<jats:sup>2<\/jats:sup>; n = 10) compared with monolayers of cells isolated from normal mice (27.3 \u00b1 3.4 \u03bcA\/cm<jats:sup>2<\/jats:sup>; n = 12). The results of these studies suggest that epithelial sodium channel-mediated sodium absorption is decreased in principal cells of ARPKD CD cysts and that the reduction in sodium absorption may contribute to the accumulation of luminal fluid.<\/jats:p>","DOI":"10.1152\/ajprenal.00169.2003","type":"journal-article","created":{"date-parts":[[2004,1,31]],"date-time":"2004-01-31T01:13:21Z","timestamp":1075511601000},"page":"F244-F254","source":"Crossref","is-referenced-by-count":47,"title":["Decreased amiloride-sensitive Na<sup>+<\/sup>absorption in collecting duct principal cells isolated from BPK ARPKD mice"],"prefix":"10.1152","volume":"286","author":[{"given":"Elias I.","family":"Veizis","sequence":"first","affiliation":[{"name":"Departments of Pediatrics and Physiology and Biophysics, Rainbow Center for Childhood PKD, Case Western Reserve University, Cleveland, Ohio 44106-4948"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cathleen R.","family":"Carlin","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Physiology and Biophysics, Rainbow Center for Childhood PKD, Case Western Reserve University, Cleveland, Ohio 44106-4948"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Calvin U.","family":"Cotton","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Physiology and Biophysics, Rainbow Center for Childhood PKD, Case Western Reserve University, Cleveland, Ohio 44106-4948"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"24","reference":[{"key":"REF1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1996.270.6.C1571"},{"key":"REF2","unstructured":"Avner EDand Sweeney WE.Apical epidermal growth factor receptor expression defines a distinct cystic tubular epithelial phenotype in autosomal recessive polycystic kidney disease (Abstract).Pediatr Res37A: 359, 1995."},{"key":"REF3","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.89.16.7447"},{"key":"REF4","unstructured":"Avner ED, Sweeney WE, and Woychik RP.Inhibition of epidermal growth factor receptor activity modulates collecting tubule cystogenesis in vitro (Abstract).J Am Soc Nephrol6: 690A, 1995."},{"key":"REF5","doi-asserted-by":"publisher","DOI":"10.1038\/ki.1978.75"},{"key":"REF6","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.16.9424"},{"key":"REF7","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.8.3.257"},{"key":"REF8","doi-asserted-by":"publisher","DOI":"10.1172\/JCI113833"},{"key":"REF9","doi-asserted-by":"publisher","DOI":"10.1007\/BF01463929"},{"key":"REF10","doi-asserted-by":"publisher","DOI":"10.1038\/ki.1993.17"},{"key":"REF11","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1994.267.6.C1682"},{"key":"REF12","doi-asserted-by":"publisher","DOI":"10.1038\/ki.1996.304"},{"key":"REF13","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.1995.269.2.H487"},{"key":"REF14","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.89.12.5487"},{"key":"REF15","doi-asserted-by":"crossref","unstructured":"Grantham JJ.Fluid secretion, cellular proliferation, and the pathogenesis of renal epithelial cysts.J Am Soc Nephrol3: 1843-1857, 1993.","DOI":"10.1681\/ASN.V3121841"},{"key":"REF16","doi-asserted-by":"publisher","DOI":"10.1007\/s004240050806"},{"key":"REF17","doi-asserted-by":"crossref","unstructured":"Kim J, Kim YH, Cha JH, Tisher CC, and Madsen KM.Intercalated cell subtypes in connecting tubule and cortical collecting duct of rat and mouse.J Am Soc Nephrol10: 1-12, 1999.","DOI":"10.1681\/ASN.V1011"},{"key":"REF18","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.86.23.9089"},{"key":"REF19","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.00122.2001"},{"key":"REF20","unstructured":"McDonald RA, Watkins SL, and Avner ED.Polycystic kidney disease. 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