Abstract
ABSTRACT: Epithelial hyperplasia is an early feature of the renal and biliary lesions in autosomal recessive polycystic kidney disease (ARPKD). To explore the cellular basis of this hyperplasia we isolated, cultured, and characterized biliary tract epithelium from common bile duct explants of mice with ARPKD (the BPK mouse) and controls. Primary cultures resulted in dense colonies of contact-inhibited epithelial cells with a homogenous growth pattern. Colony growth in serum-free basal medium (BM) of BPK-derived cells was not different from controls. Supplementation of BM with epidermal growth factor (EGF) induced a proliferative response in BPK-derived cells that was significantly increased over controls as assessed by [3H]thymidine uptake and expressed as percent change over growth in BM (BPK 239% and controls 131% of BM growth). In contrast, no differences between BPK- and control-derived cells were found with regard to the effects of BM supplementation with IGF-I, IGF-II, acidic fibroblast growth factor, keratinocyte growth factor, hepatocyte growth factor, or transforming growth factor-β. Primary culture of biliary epithelium may provide a useful in vitro model for the study of the cellular pathophysiology of ARPKD. Our data demonstrate that increased epithelial sensitivity to EGF-like proteins may play a role in biliary epithelial proliferative changes which parallel renal tubular epithelial proliferation in ARPKD.
Similar content being viewed by others
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Nauta, J., Sweeney, W., Rutledge, J. et al. Biliary Epithelial Cells from Mice with Congenital Polycystic Kidney Disease Are Hyperresponsive to Epidermal Growth Factor. Pediatr Res 37, 755–763 (1995). https://doi.org/10.1203/00006450-199506000-00014
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1203/00006450-199506000-00014
This article is cited by
-
Pathophysiology of childhood polycystic kidney diseases: new insights into disease-specific therapy
Pediatric Research (2014)
-
A novel method for establishment and characterization of extrahepatic bile duct epithelial cells from mice
In Vitro Cellular & Developmental Biology - Animal (2010)
-
Diagnosis, Pathogenesis, and Treatment Prospects in Cystic Kidney Disease
Molecular Diagnosis & Therapy (2006)
-
Molecular and cellular pathophysiology of autosomal recessive polycystic kidney disease (ARPKD)
Cell and Tissue Research (2006)