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Nephrol Dial Transplant. 2017 Aug 29. doi: 10.1093/ndt/gfx262. [Epub ahead of print]

Targeting new cellular disease pathways in autosomal dominant polycystic kidney disease.

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Kidney Research Center, Department of Nephrology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.
Kidney Genetics Group, Academic Nephrology Unit, University of Sheffield Medical School, Sheffield, UK.
Sheffield Kidney Institute, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.


Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited cause of end-stage renal failure. Understanding the molecular and cellular pathogenesis of ADPKD could help to identify new targets for treatment. The classic cellular cystic phenotype includes changes in proliferation, apoptosis, fluid secretion, extracellular matrix and cilia function. Hoever, recent research, suggests that the cellular cystic phenotype could be broader and that changes, such as altered metabolism, autophagy, inflammation, oxidative stress and epigenetic modification, could play important roles in the processes of cyst initiation, cyst growth or disease progression. Here we review these newer cellular pathways, describe evidence for their possible links to cystic pathogenesis or different stages of disease and discuss the options for developing novel treatments.


ADPKD; PKD1; PKD2; pathogenesis; polycystic kidney disease; treatment


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