Hereditary E200K mutation within the prion protein gene alters human iPSC derived cardiomyocyte function
Abstract 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 dir...
Main Authors: | Aleksandar R. Wood, Simote T. Foliaki, Bradley R. Groveman, Ryan O. Walters, Katie Williams, Jue Yuan, Wen-Quan Zou, Cathryn L. Haigh |
---|---|
Format: | Article |
Language: | English |
Published: |
Nature Portfolio
2022-09-01
|
Series: | Scientific Reports |
Online Access: | https://doi.org/10.1038/s41598-022-19631-5 |
Similar Items
-
Pathogenic Prion Protein Isoforms Are Not Present in Cerebral Organoids Generated from Asymptomatic Donors Carrying the E200K Mutation Associated with Familial Prion Disease
by: Simote T. Foliaki, et al.
Published: (2020-06-01) -
Prion propagation and cellular dysfunction in prion disease: Disconnecting the dots.
by: Simote T Foliaki, et al.
Published: (2023-10-01) -
Prion propagation and cellular dysfunction in prion disease: Disconnecting the dots
by: Simote T. Foliaki, et al.
Published: (2023-10-01) -
Temporary alteration of neuronal network communication is a protective response to redox imbalance that requires GPI-anchored prion protein
by: Simote T. Foliaki, et al.
Published: (2023-07-01) -
Reduced SOD2 expression does not influence prion disease course or pathology in mice
by: Simote T. Foliaki, et al.
Published: (2021-01-01)