Modeling reduced contractility and impaired desmosome assembly due to plakophilin-2 deficiency using isogenic iPS cell-derived cardiomyocytes

Loss-of-function mutations in PKP2, which encodes plakophilin-2, cause arrhythmogenic cardiomyopathy (AC). Restoration of deficient molecules can serve as upstream therapy, thereby requiring a human model that recapitulates disease pathology and provides distinct readouts in phenotypic analysis for...

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Detalles Bibliográficos
Autores principales: Inoue, Hiroyuki, Nakamura, Satoki, Higo, Shuichiro, Shiba, Mikio, Kohama, Yasuaki, Kondo, Takumi, Kameda, Satoshi, Tabata, Tomoka, Okuno, Shota, Ikeda, Yoshihiko, Li, Junjun, Liu, Li, Yamazaki, Satoru, Takeda, Maki, Ito, Emiko, Takashima, Seiji, Miyagawa, Shigeru, Sawa, Yoshiki, Hikoso, Shungo, Sakata, Yasushi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8828557/
https://www.ncbi.nlm.nih.gov/pubmed/35063130
http://dx.doi.org/10.1016/j.stemcr.2021.12.016

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