Faculty Collaboration Database

Epithelial polarity and differentiation in polycystic kidney disease. J Cell Sci Suppl 1993;17:217-22

Date

01/01/1993

Pubmed ID

8144700

DOI

10.1242/jcs.1993.supplement_17.30

Scopus ID

2-s2.0-0027754746 (requires institutional sign-in at Scopus site)   36 Citations

Abstract

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's 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 basolateral 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.

Author List

Avner ED

Author

Ellis D. Avner MD Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Animals
Cell Differentiation
Cell Polarity
Disease Models, Animal
Epithelium
Humans
Kidney Tubules, Collecting
Kidney Tubules, Proximal
Mice
Mice, Inbred C57BL
Mice, Mutant Strains
Polycystic Kidney, Autosomal Recessive
Sodium-Potassium-Exchanging ATPase