DLG1 functions upstream of SDCCAG3 and IFT20 to control ciliary targeting of polycystin-2

Abstract

(Figure presented.) The Scribble polarity complex protein DLG1 regulates ciliary length and protein composition in kidney epithelial cells. A Congenital Anomalies of the Kidney and Urinary Tract (CAKUT)-associated DLG1 variant may be associated with ciliary dysfunction. Loss of DLG1 causes ciliary elongation in kidney epithelial cells. Loss of DLG1 impairs targeting of SDCCAG3, IFT20 and PC2 to the primary cilium of kidney epithelial cells. The CAKUT-associated p.T489R DLG1 fails to rescue ciliary defects of Dlg1-/- cells, indicating a possible ciliary involvement in CAKUT disease etiology.