Altered gene expression and DNA damage in peripheral blood cells from Friedreich's ataxia patients: cellular model of pathology.

The neurodegenerative disease Friedreich's ataxia (FRDA) is the most common autosomal-recessively inherited ataxia and is caused by a GAA triplet repeat expansion in the first intron of the frataxin gene. In this disease, transcription of frataxin, a mitochondrial protein involved in iron homeo...

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Bibliographic Details
Main Authors: Astrid C Haugen, Nicholas A Di Prospero, Joel S Parker, Rick D Fannin, Jeff Chou, Joel N Meyer, Christopher Halweg, Jennifer B Collins, Alexandra Durr, Kenneth Fischbeck, Bennett Van Houten
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2010-01-01
Series:PLoS Genetics
Online Access:http://europepmc.org/articles/PMC2799513?pdf=render