Reduction of elevated Gli3 does not alter the progression of autosomal recessive polycystic kidney disease

Reduction of elevated Gli3 does not alter the progression of autosomal recessive polycystic kidney disease

Abstract Polycystic kidney diseases (PKD) are genetic disorders which disrupt kidney architecture and function. Autosomal recessive PKD (ARPKD) is a rare form of PKD, caused by mutations in PKHD1, and clinically more severe than the more common autosomal dominant PKD (ADPKD). Prior studies have impl...

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Main Authors: Lauren G. Russell, Maria Kolatsi‐Joannou, Laura Wilson, Jennifer C. Chandler, Nuria Perretta Tejedor, Georgie Stagg, Karen L. Price, Christopher J. Rowan, Tessa Crompton, Norman D. Rosenblum, Paul J. D. Winyard, David A. Long
Format: Article
Language:English
Published: Wiley 2025-01-01
Series:Physiological Reports
Subjects:
autosomal recessive polycystic kidney disease
cystogenesis
hedgehog signaling
human model
mouse model
Online Access:https://doi.org/10.14814/phy2.70191
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