Article
Hereditary E200K mutation within the prion protein gene alters human iPSC derived cardiomyocyte function.
Scientific reports - 22 Sept 2022
Wood Aleksandar R, Foliaki Simote T, Groveman Bradley R, Walters Ryan O, Williams Katie, Yuan Jue, Zou Wen-Quan, Haigh Cathryn L
Abstract excerpt
Cardiomyopathy is a co-morbidity of some prion diseases including genetic disease caused by mutations within the PrP gene (PRNP). Although the cellular prion protein (PrP) has been shown to protect against cardiotoxicity caused by oxidative stress, it is unclear if the cardiomyopathy is directly linked to PrP dysfunction. We differentiated cardiomyocyte cultures from donor human induced pluripotent stem cells and...
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