ADPKD cell proliferation and Cl--dependent fluid secretion

• Published in: Methods in Kidney Cell Biology Part A Vol. 153; pp. 69 - 92
• Main Authors: Reif, Gail A, Wallace, Darren P
• Format: Book Chapter Journal Article
• Language: English
• Published: United States Elsevier Science & Technology 2019
• Subjects: Biological Transport - physiology; Cell Count - instrumentation; Cell Count - methods; Cell Proliferation; Chlorides - metabolism; Epithelial Cells - physiology; Humans; Kidney - cytology; Kidney - metabolism; Kidney - pathology; Polycystic Kidney, Autosomal Dominant - pathology; Primary Cell Culture - instrumentation; Primary Cell Culture - methods; Ion transport; In vitro models; Cyst growth; Anion secretion; Autosomal dominant polycystic kidney disease
• ISBN: 0128170824; 9780128170823
• ISSN: 0091-679X
• DOI: 10.1016/bs.mcb.2019.06.001

Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder characterized by bilateral fluid-filled cysts, renal inflammation and extensive fibrosis, leading to the progressive decline in kidney function. Renal cyst formation begins in utero from aberrant proliferation of tubule epithelial cells; however, the mechanisms for cystogenesis remain unclear. Cell proliferation and Cl
-dependent fluid secretion, which drives the accumulation of cyst fluid, are responsible for inexorable growth of cysts and the remarkable appearance of massively enlarged ADPKD kidneys. Investigators have used in vitro assays to explore cellular and molecular mechanisms involved in ADPKD cyst epithelial cell proliferation and Cl
-dependent fluid secretion in experimentally controlled environments. These assays have been used to evaluate potential therapeutic approaches to inhibit cellular pathways involved in cyst growth. This chapter discusses methods for measuring ADPKD cell proliferation, transepithelial Cl
secretion, and net fluid transport across cyst epithelial cell monolayers.