The chloride channel CFTR is not required for cyst growth in an ADPKD mouse model

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the development of bilateral renal cysts which enlarge continuously, leading to compression adjacent intact nephrons. The growing lead a progressive decline in function. Cyst growth driven enhanced cell proliferation and chloride secretion into cyst lumen. Chloride believed occur mainly cAMP-activated cystic fibrosis transmembrane conductance regulator (CFTR), with some contribution calcium-activated channel TMEM16A. However, our previous work suggested TMEM16A as major factor for formation. CFTR formation has never been demonstrated an adult ADPKD mouse model. We used mice inducible tubule-specific Pkd1 knockout, consistently develop kidneys upon deletion Pkd1. Cellular properties, ion currents, these were compared that carrying co-deletion Cftr. Knockout Cftr did not reveal any significant impact on Furthermore, knockout attenuate largely augmented observed kidneys. Patch clamp analysis primary epithelial cells lacking expression indicated only marginal whole Cl? clearly dominated currents. In conclusion, does essentially contribute caused Enhanced primarily upregulation