Article
Mechanism of Fibrosis in HNF1B-Related Autosomal Dominant Tubulointerstitial Kidney Disease.
Journal of the American Society of Nephrology : JASN - 1 Oct 2018
Chan Siu Chiu, Zhang Ying, Shao Annie, Avdulov Svetlana, Herrera Jeremy, Aboudehen Karam, Pontoglio Marco, Igarashi Peter
Abstract excerpt
BACKGROUND: Mutation of HNF1B, the gene encoding transcription factor HNF-1β, is one cause of autosomal dominant tubulointerstitial kidney disease, a syndrome characterized by tubular cysts, renal fibrosis, and progressive decline in renal function. HNF-1β has also been implicated in epithelial-mesenchymal transition (EMT) pathways, and sustained EMT is associated with tissue fibrosis. The mechanism whereby...
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