Mechanism of Fibrosis in HNF1B-Related Autosomal Dominant Tubulointerstitial Kidney Disease

Abstract

Ablation of HNF-1<i>β</i> in renal epithelial cells leads to the activation of a Twist2-dependent transcriptional network that induces EMT and aberrant TGF-<i>β</i> signaling, resulting in renal fibrosis through a cell-nonautonomous mechanism.