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PROTEIN FOLDING AND DISEASE THE MITOCHONDRIAL PROTEIN FRATAXIN
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| 6.69 MB | Adobe PDF |
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This dissertation focuses on the study of frataxin, a small
mitochondrial protein whose deficiency is associated with the
neurodegenerative disease Friedreich's ataxia (FRDA). Aiming at a
better understanding of frataxin conformational and functional
properties, two lines of research were followed: first, the effect of
FRDA-related mutations in human frataxin (FXN) were studied and the
role of oxidative stress related modification addressed; second, yeast
frataxin (Yfh1) orthologue was used to explore the conformational and
functional properties of the protein.(...)