XBP1 Activation Reduces Severity of Polycystic Kidney Disease due to a Nontruncating

Autor: Matteus, Krappitz, Rishi, Bhardwaj, Ke, Dong, Tobias, Staudner, Duygu Elif, Yilmaz, Carlotta, Pioppini, Parisa, Westergerling, David, Ruemmele, Till, Hollmann, Thuy Anh, Nguyen, Yiqiang, Cai, Anna-Rachel, Gallagher, Stefan, Somlo, Sorin, Fedeles
Rok vydání: 2021
Zdroj: Journal of the American Society of Nephrology : JASN.
ISSN: 1533-3450
Popis: Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations inWe engineered aExpression of active XBP1 in cultured cells bearing PC1Modulating ER chaperone function through XBP1 activity improved Pkd in a murine model of PC1, suggesting therapeutic targeting of hypomorphic mutations.
Databáze: OpenAIRE