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- Title
New Insights into the Molecular Mechanisms Targeting Tubular Channels/Transporters in PKD Development.
- Authors
Ming Wu; Shengqiang Yu
- Abstract
Background: Autosomal dominant polycystic kidney disease (PKD) or autosomal recessive PKD is caused by a mutation in the PKD1, PKD2 or PKHD1 gene, which encodes polycystin-1, polycystin-2 orfibrocystin, respectively. Embryonic and postnatal mutation studies showthattransportorchannel 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. Summary: 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. Key Messages: Abnormalities in calcium handling and exuberant cAMPdependent cystic fibrosistransmembrane conductance regulator-mediated fluid secretion in the collecting duct are the most important issues in the pathogenesis of PKD.
- Publication
Kidney Diseases, 2016, Vol 2, Issue 3, p128
- ISSN
2296-9381
- Publication type
Article
- DOI
10.1159/000444839