Friedreich’s ataxia (FRDA), an autosomal recessive neurodegenerative disease caused by a defect in the gene for the mitochondrial protein frataxin, also displays cardiac involvement, most commonly as dilated cardiomyopathy and arrhythmia. Progression is related to the amount of residual frataxin and disease duration. Mechanical cardiomyocyte stress is very high in the left ventricular wall and may be responsible for the extensive scarring and remodeling of the myocardium.

Read More: Cardiac remodeling of intercalated discs in Friedreich's ataxia