SOX9-Dependent Fibrosis Drives Renal Function in Nephronophthisis

Researchers showed that kidney-specific deletion of Fbxw7, the recognition receptor of the SCFFBW7 E3 ubiquitin ligase, resulted in a juvenile-adult nephronophthisis-like pathology characterized by slow-progressing corticomedullary cysts, tubular degeneration, and gradual loss of kidney function.