M. Afshani

CFTR correctors suppress Ca²⁺-cAMP signaling and cyst growth in primary cultures of ARPKD cholangiocytes.

Autosomal recessive polycystic kidney disease (ARPKD) is a severe ciliopathy caused by mutations in Polycystic kidney and hepatic disease 1 gene (PKHD1), resulting in progressive renal and hepatobiliary disease for which effective targeted therapies are lacking. Although reduced cystic fibrosis transmembrane conductanc...