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Fluid transport and cystogenesis in autosomal dominant polycystic kidney disease

par Terryn, Sara;Ho, Anh;Beauwens, Renaud ;Devuyst, Olivier
Référence Biochimica et biophysica acta. Molecular basis of disease, 1812, 10, page (1314-1321)
Publication Publié, 2011-10

Article révisé par les pairs

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Résumé :

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent inherited nephropathy. The development and enlargement of cysts in ADPKD requires tubular cell proliferation, abnormalities in the extracellular matrix and transepithelial fluid secretion. Multiple studies have suggested that fluid secretion across ADPKD cyst-lining cells is driven by the transepithelial secretion of chloride, mediated by the apical CFTR channel and specific basolateral transporters. The whole secretory process is stimulated by increased levels of cAMP in the cells, probably reflecting modifications in the intracellular calcium homeostasis and abnormal stimulation of the vasopressin V2 receptor. This review will focus on the pathophysiology of fluid secretion in ADPKD cysts, starting with classic, morphological and physiological studies that were followed by investigations of the molecular mechanisms involved and therapeutic trials targeting these pathways in cellular and animal models and ADPKD patients. © 2011 Elsevier B.V.