title: Polycystic kidney disease: pathogenesis and potential therapies.
abstract: 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.
authors.affiliation: Department of Cellular and Molecular Physiology, Yale University School of Medicine, New Haven, CT, USA.
authors.forename: Vinita
authors.initials: V
authors.lastname: Takiar
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Caplan
mesh_terms: D000818:Animals; D002923:Cilia / Q000473:pathology / Q000502:physiology; D000242:Cyclic AMP / Q000037:antagonists & inhibitors / Q000502:physiology; D020420:Cyst Fluid / Q000378:metabolism; D006801:Humans; D016891:Polycystic Kidney, Autosomal Dominant / Q000188:drug therapy* / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D015398:Signal Transduction; D050396:TRPP Cation Channels / Q000235:genetics / Q000502:physiology
chemical_list: D000242:Cyclic AMP; D050396:TRPP Cation Channels