The Progression of Cardiomyopathy in the Mitochondrial Disease, Friedreich’s Ataxia

摘要: Friedreich’s ataxia (FA) is a debilitating mitochondrial disorder leading to neural and cardiac degeneration that caused by mutation in the frataxin gene. The most common cause of death these patients heart failure, although it not known how deficiency potentiates observed cardiomyopathy. However, there have been number proposed mechanisms involving function frataxin, origins failure FA will be discussed this chapter. It hoped elucidation potential lead comprehensive understanding pathogenesis FA, contribute development treatments therapeutics.