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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Pubblicato in:PLoS One
Autori principali: Chutake, Yogesh K., Costello, Whitney N., Lam, Christina C., Parikh, Aniruddha C., Hughes, Tamara T., Michalopulos, Michael G., Pook, Mark A., Bidichandani, Sanjay I.
Natura: Artigo
Lingua:Inglês
Pubblicazione: Public Library of Science 2015
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Research Article
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC4579136/
https://ncbi.nlm.nih.gov/pubmed/26393353
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0138437
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