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Diverse effects in Friedreich's ataxia place PGC-1alpha center-stage

Friedreich's ataxia (FRDA), the most common inherited ataxia, is characterized by focal neurodegeneration, diabetes mellitus and life-threatening cardiomyopathy. Frataxin, which is significantly reduced in patients with this recessive disorder, is a mitochondrial iron-binding protein, but how its deficiency leads to neurodegeneration and metabolic derangements is not known.

Read More: Diverse effects in Friedreich's ataxia place PGC-1α center-stage

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