Activated Hedgehog-GLI Signaling Causes Congenital Ureteropelvic Junction Obstruction.

ABSTRACT: Intrinsic ureteropelvic junction obstruction is the most common cause of congenital hydronephrosis, yet the underlying pathogenesis is undefined. Hedgehog proteins control morphogenesis by promoting GLI-dependent transcriptional activation and inhibiting the formation of the GLI3 transcriptional repressor. Hedgehog regulates differentiation and proliferation of ureteric smooth muscle progenitor cells during murine kidney-ureter development. Histopathologic findings of smooth muscle cell hypertrophy and stroma-like cells, consistently observed in obstructing tissue at the time of surgical correction, suggest that Hedgehog signaling is abnormally regulated during the genesis of congenital intrinsic ureteropelvic junction obstruction. Here, we demonstrate that constitutively active Hedgehog s

SUBMITTER: Sheybani-Deloui S 

PROVIDER: S-EPMC5791051 | biostudies-literature | 2018 Feb

REPOSITORIES: biostudies-literature