Epigenetic silencing in Friedreich ataxia is associated with depletion of CTCF (CCCTC-binding factor) and antisense transcription.

BACKGROUND:Over 15 inherited diseases are caused by expansion of triplet-repeats. Friedreich ataxia (FRDA) patients are homozygous for an expanded GAA triplet-repeat sequence in intron 1 of the FXN gene. The expanded GAA triplet-repeat results in deficiency of FXN gene transcription, which is revers...

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Bibliographic Details
Main Authors: Irene De Biase, Yogesh K Chutake, Paul M Rindler, Sanjay I Bidichandani
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2009-11-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC2780319?pdf=render