A novel GAA-repeat-expansion-based mouse model of Friedreich’s ataxia
Friedreich’s ataxia (FRDA) is an autosomal recessive neurodegenerative disorder caused by a GAA repeat expansion mutation within intron 1 of the FXN gene, resulting in reduced levels of frataxin protein. We have previously reported the generation of human FXN yeast artificial chromosome (YAC) transg...
Main Authors: | , , , , , , |
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Format: | Article |
Language: | English |
Published: |
The Company of Biologists
2015-03-01
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Series: | Disease Models & Mechanisms |
Subjects: | |
Online Access: | http://dmm.biologists.org/content/8/3/225 |