title: New Insights into the Molecular Mechanisms Targeting Tubular Channels/Transporters in PKD Development.
abstract: BACKGROUND
  Autosomal dominant polycystic kidney disease (PKD) or autosomal recessive PKD is caused by a mutation in thePKD1,PKD2orPKHD1gene, which encodes polycystin-1, polycystin-2 or fibrocystin, respectively. Embryonic and postnatal mutation studies show that transport or channel function is dysregulated before the initiation of cystogenesis, suggesting that the abnormality of transport or channel function plays a critical role in the pathology of PKD.
  
  
  CONCLUSIONS
  Polycystin-2 by itself is a calcium-permeable cation channel, and its channel function can be regulated by polycystin-1 or fibrocystin. In this paper, we reviewed the current knowledge about calcium transports and cyclic adenosine monophosphate (cAMP)-driven chloride transports in PKD. In addition, the function and the underlining mechanism of glucose transporters, phosphate transporters and water channels in PKD are also discussed.
  
  
  CONCLUSIONS
  Abnormalities in calcium handling and exuberant cAMP-dependent cystic fibrosis transmembrane conductance regulator-mediated fluid secretion in the collecting duct are the most important issues in the pathogenesis of PKD.
authors.affiliation: Kidney Institute of PLA, Division of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, PR China.
authors.forename: Ming
authors.initials: M
authors.lastname: Wu
authors.affiliation: Kidney Institute of PLA, Division of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, PR China.
authors.forename: Shengqiang
authors.initials: S
authors.lastname: Yu
keywords: Channels; Molecular mechanism; Polycystic kidney disease; Transporters