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Acute frataxin knockdown in induced pluripotent stem cell-derived cardiomyocytes activates a type I interferon response

Friedreich ataxia, the most common hereditary ataxia, is a neuro- and cardio-degenerative disorder caused, in most cases, by decreased expression of the mitochondrial protein frataxin. Cardiomyopathy is the leading cause of premature death. Frataxin functions in the biogenesis of iron-sulfur cluster...

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Detalles Bibliográficos
Autores principales: Cotticelli, M. Grazia, Xia, Shujuan, Truitt, Rachel, Doliba, Nicolai M., Rozo, Andrea V., Tobias, John W., Lee, Taehee, Chen, Justin, Napierala, Jill S., Napierala, Marek, Yang, Wenli, Wilson, Robert B.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9637271/
https://www.ncbi.nlm.nih.gov/pubmed/36107856
http://dx.doi.org/10.1242/dmm.049497