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Article

XBP1 Activation Reduces Severity of Polycystic Kidney Disease due to a Nontruncating Polycystin-1 Mutation in Mice.

Journal of the American Society of Nephrology : JASN - 1 Jan 2023

Krappitz Matteus, Bhardwaj Rishi, Dong Ke, Staudner Tobias, Yilmaz Duygu Elif, Pioppini Carlotta, Westergerling Parisa, Ruemmele David, Hollmann Till, Nguyen Thuy Anh, Cai Yiqiang, Gallagher Anna-Rachel, Somlo Stefan, Fedeles Sorin

Abstract excerpt

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in Pkd1 and Pkd2. They encode the polytopic integral membrane proteins polycystin-1 (PC1) and polycystin-2 (PC2), respectively, which are expressed on primary cilia. Formation of kidney cysts in ADPKD starts when a somatic second hit mechanism inactivates the wild-type Pkd allele. Approximately one quarter of families with...

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Identifiers

PMID: 36270750
DOI: 10.1681/ASN.2021091180
PMCID: PMC10101557

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