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The role of oxidative stress in Friedreich's ataxia

Oxidative stress and increase in the levels of free radicals are important markers associated with several pathologies, including Alzheimer's disease, cancer and diabetes. Friedreich's ataxia is an excellent paradigmatic example of a disease in which oxidative stress plays an important, albeit not completely understood, role.

Friedreich's ataxia is a rare genetic neurodegenerative disease which involves partial silencing of frataxin, a small mitochondrial protein completely ignored before being linked to Friedreich's ataxia. More than twenty years later, we now know how important this protein is, being essential and part of the vital machinery which produces iron-sulfur clusters in the cell.

In this Review, we revisit the most important steps which have brought us to our current understanding of the function of frataxin and its role in disease. We discuss the current hypotheses on the role of oxidative stress in Friedreich's ataxia, reviewing some of the existing animal and cellular models. We also evaluate new techniques which can assist in the study of the disease mechanisms and in understanding the interplay between primary and secondary phenotypes.

Read the entire article HERE


About the Author

Jen Farmer

Jen Farmer

Executive Director

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