Time-resolved functional analysis of acute impairment of frataxin expression in an inducible cell model of Friedreich ataxia

Time-resolved functional analysis of acute impairment of frataxin expression in an inducible cell model of Friedreich ataxia

Friedreich ataxia is a neurodegenerative disease caused by a GAA triplet repeat expansion in the first intron of the frataxin gene, which results in reduced expression levels of the corresponding protein. Despite numerous animal and cellular models, therapeutic options that mechanistically address i...

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Bibliographic Details
Main Authors: Dörte Poburski, Josefine Barbara Boerner, Michel Koenig, Michael Ristow, René Thierbach
Format: Article
Language:English
Published: The Company of Biologists 2016-05-01
Series:Biology Open
Subjects:
Frataxin
Friedreich ataxia
Mammalian cell model
Iron sulfur cluster biosynthesis
ROS
HTS
Online Access:http://bio.biologists.org/content/5/5/654

Internet

http://bio.biologists.org/content/5/5/654

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