Article
Molecular and Cellular Substrates for the Friedreich Ataxia. Significance of Contactin Expression and of Antioxidant Administration.
Molecules (Basel, Switzerland) - 7 Sept 2020
Bizzoca Antonella, Caracciolo Martina, Corsi Patrizia, Magrone Thea, Jirillo Emilio, Gennarini Gianfranco
Abstract excerpt
In this study, the neural phenotype is explored in rodent models of the spinocerebellar disorder known as the Friedreich Ataxia (FA), which results from mutations within the gene encoding the Frataxin mitochondrial protein. For this, the M12 line, bearing a targeted mutation, which disrupts the Frataxin gene exon 4 was used, together with the M02 line, which, in addition, is hemizygous for the human Frataxin gene...
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