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Am J Physiol Cell Physiol 281: C1695-C1705, 2001;
0363-6143/01 $5.00
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Vol. 281, Issue 5, C1695-C1705, November 2001

Phenotypic analysis of conditionally immortalized cells isolated from the BPK model of ARPKD

William E. Sweeney Jr.1,*, Linda Kusner2,*, Cathleen R. Carlin2,3, Sharon Chang1, Lidia Futey1, Calvin U. Cotton1,2, Katherine MacRae Dell1, and Ellis D. Avner1

1 Rainbow Center for Childhood PKD, Department of Pediatrics, Rainbow Babies and Children's Hospital and Case Western Reserve University, Cleveland; and 2 Department of Physiology and Biophysics, 3 Case Western Reserve University Cancer Center, Cleveland, Ohio, 44106

To study the pathophysiology of autosomal recessive polycystic kidney disease (ARPKD), we sought to develop conditionally immortalized control and cystic murine collecting tubule (CT) cell lines. CT cells were isolated from intercross breedings between BPK mice (bpk+/-), a murine model of ARPKD, and the Immorto mice (H-2Kb-ts-A58+/+). Second-generation outbred offspring (BPK × Immorto) homozygous for the BPK mutation (bpk-/-; Im+/±; cystic BPK/H-2Kb-ts-A58), were phenotypically indistinguishable from inbred cystic BPK animals (bpk-/-). Cystic BPK/H-2Kb-ts-A58 mice developed biliary ductal ectasia and massively enlarged kidneys, leading to renal failure and death by postnatal day 24. Principal cells (PC) were isolated from outbred cystic and noncystic BPK/H-2Kb-ts-A58 littermates at specific developmental stages. Epithelial monolayers were under nonpermissive conditions for markers of epithelial cell polarity and PC function. Cystic and noncystic cells displayed several properties characteristic of PCs in vivo, including amiloride-sensitive sodium transport and aquaporin 2 expression. Cystic cells exhibited apical epidermal growth factor receptor (EGFR) mislocalization but normal expression of ZO-1 and E-cadherin. Hence, these cell lines retain the requisite characteristics of PCs, and cystic BPK/H-2Kb-ts-A58 PCs retained the abnormal EGFR membrane expression characteristic of ARPKD. These cell lines represent important new reagents for studying the pathogenesis of ARPKD.

epidermal growth factor receptor, principal cells, cystic kidney

* W. E. Sweeney, Jr., and L. Kusner contributed equally to this work.




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