Regenerative Therapy in Autosomal Recessive Polycystic Kidney Disease

¹University of Cologne, Deutz-Kalker Str. 118, 50679, Cologne, Germany.

Corresponding Author:Mohamed SA Mohamed, University of Cologne, Deutz-Kalker Str. 118, 50679, Cologne, Germany,Tel: +49 221 8275-2456; Email: [email protected]

Received Date: 13 Jun 2016   

Accepted Date: 17 Jun 2016   

Published Date: 20 Jun 2016

Copyright © 2016 Mohamed MSA

Citation: Mohamed MSA. (2016). Regenerative Therapy in Autosomal Recessive Polycystic Kidney Disease. Mathews J Surgery. 1(1): 002.

ABSTRACT

Autosomal recessive polycystic kidney disease (ARPKD) is a developmental disease that results from fibrocystin loss of function mutation. It affects mainly renal collecting ducts and biliary system. The exact mechanism of how fibrocystin mutation results in the disease manifestations is not known as the exact functions of fibrocystin are not very well identified. One of the mechanisms involved in the development of ARPKD is the impaired eipthelial cell polarity, which renders the epithelium secretory rather than absorptive. 
This paper sheds the spot on a hypothesis that the impaired epithelial cell polarity seen in ARPKD might result from the impaired β₁β₂ Na⁺/K⁺ ATPase expression together with the impaired focal adhesion kinase (FAK) activation and function, with the possible involvement of the adhesion signalling in the development of those interactions. Restoration of the functions of FAK and β₁ Na⁺/K⁺ATPase in the renal and biliary epithelia of ARPKD patients might be a future hope of recovery that could be achieved through the regenerative therapy, using stem cells that express myroslated FAK and β₁ Na⁺/K⁺ ATPase subunit to slowdown or reverse cyst formation.

KEYWORDS

Arpkd; Fibrocystin; Fak; Na⁺/K⁺ ATPase; Stem Cell Therapy and Epithelial Cell Polarity.