Article
'Mitochondrial energy imbalance and lipid peroxidation cause cell death in Friedreich's ataxia'.
Cell death & disease - 26 May 2016
Abeti R, Parkinson M H, Hargreaves I P, Angelova P R, Sandi C, Pook M A, Giunti P, Abramov A Y
Abstract excerpt
Friedreich's ataxia (FRDA) is an inherited neurodegenerative disease. The mutation consists of a GAA repeat expansion within the FXN gene, which downregulates frataxin, leading to abnormal mitochondrial iron accumulation, which may in turn cause changes in mitochondrial function. Although, many studies of FRDA patients and mouse models have been conducted in the past two decades, the role of frataxin in...
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