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Selected Histone Deacetylase Inhibitors Reverse the Frataxin Transcriptional Defect in a Novel Friedreich’s Ataxia Induced Pluripotent Stem Cell-Derived Neuronal Reporter System

Friedreich’s ataxia (FRDA) is a neurodegenerative disorder caused by the expansion of guanine–adenine–adenine repeats within the first intron of the frataxin (FXN) gene. The location and nature of the expansion have been proven to contribute to transcriptional repression of FXN by decreasing the rat...

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Detalles Bibliográficos
Autores principales: Schreiber, Anna M., Li, Yanjie, Chen, Yi-Hsien, Napierala, Jill S., Napierala, Marek
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8904878/
https://www.ncbi.nlm.nih.gov/pubmed/35281493
http://dx.doi.org/10.3389/fnins.2022.836476