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As an attempt to generate a mouse model of the disease, we introduced a (GAA)<jats:sub>230<\/jats:sub> repeat within the mouse frataxin gene by homologous recombination. GAA repeat knockin mice were crossed with frataxin knockout mice to obtain double heterozygous mice expressing 25\u201336% of wild\u2010type frataxin levels. These mice were viable and did not develop anomalies of motor coordination, iron metabolism or response to iron loading. 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