Article
Frataxin knockin mouse.
FEBS letters - 13 Feb 2002
Miranda Carlos J, Santos Manuela M, Ohshima Keiichi, Smith Julie, Li Liangtao, Bunting Michaeline, Cossée Mireille, Koenig Michael, Sequeiros Jorge, Kaplan Jerry, Pandolfo Massimo
Abstract excerpt
Friedreich ataxia is the consequence of frataxin deficiency, most often caused by a GAA repeat expansion in intron 1 of the corresponding gene. Frataxin is a mitochondrial protein involved in iron homeostasis. As an attempt to generate a mouse model of the disease, we introduced a (GAA)(230) repeat within the mouse frataxin gene by homologous recombination. GAA repeat knockin mice were crossed with frataxin...
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