Loss of polycystin-1 in human cyst-lining epithelia leads to ciliary dysfunction

SM Nauli, S Rossetti, RJ Kolb, FJ Alenghat… - Journal of the …, 2006 - journals.lww.com
A “two-hit” hypothesis predicts a second somatic hit, in addition to the germline mutation, as
a prerequisite to cystogenesis and has been proposed to explain the focal nature for renal
cyst formation in autosomal dominant polycystic kidney disease (ADPKD). It was reported
previously that Pkd1 null/null mouse kidney epithelial cells are unresponsive to flow
stimulation. This report shows that Pkd1+/null cells are capable of responding to mechanical
flow stimulation by changing their intracellular calcium concentration in a manner similar to …