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FXN Promoter Silencing in the Humanized Mouse Model of Friedreich Ataxia

BACKGROUND: Friedreich ataxia is caused by an expanded GAA triplet-repeat sequence in intron 1 of the FXN gene that results in epigenetic silencing of the FXN promoter. This silencing mechanism is seen in patient-derived lymphoblastoid cells but it remains unknown if it is a widespread phenomenon af...

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Detalles Bibliográficos
Autores principales:	Chutake, Yogesh K., Costello, Whitney N., Lam, Christina C., Parikh, Aniruddha C., Hughes, Tamara T., Michalopulos, Michael G., Pook, Mark A., Bidichandani, Sanjay I.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Public Library of Science 2015
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4579136/ https://www.ncbi.nlm.nih.gov/pubmed/26393353 http://dx.doi.org/10.1371/journal.pone.0138437

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