Polycystic kidney disease: Pathogenesis and potential therapies

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

卷:1812

Review

Takiar, Vinita¹  Caplan, Michael J.¹ 

[1] Yale Univ, Sch Med, Dept Cellular & Mol Physiol, New Haven, CT 06520 USA

关键词: Polycystic kidney disease;    Cilium;    Proliferation;    Signaling;   

DOI  :  10.1016/j.bbadis.2010.11.014

来源: Elsevier

【 摘 要 】

Autosomal dominant polycystic kidney disease (ADPKD) is a prevalent, inherited condition for which there is currently no effective specific clinical therapy. The disease is characterized by the progressive development of fluid-filled cysts derived from renal tubular epithelial cells which gradually compress the parenchyma and compromise renal function. Current interests in the field focus on understanding and exploiting signaling mechanisms underlying disease pathogenesis as well as delineating the role of the primary cilium in cystogenesis. This review highlights the pathogenetic pathways underlying renal cyst formation as well as novel therapeutic targets for the treatment of PKD. This article is part of a Special Issue entitled: Polycystic Kidney Disease. (C) 2010 Elsevier B.V. All rights reserved.

【 授权许可】

Free