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

Matteus KrappitzRishi BhardwajKe DongTobias StaudnerDuygu Elif YilmazCarlotta PioppiniParisa WestergerlingDavid RuemmeleTill HollmannThuy Anh NguyenYiqiang CaiAnna-Rachel GallagherStefan SomloSorin Fedeles
Published in: Journal of the American Society of Nephrology : JASN (2022)
Modulating ER chaperone function through XBP1 activity improved Pkd in a murine model of PC1, suggesting therapeutic targeting of hypomorphic mutations.
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