Friedreich's ataxia (FRDA) is a hereditary neuromuscular disorder. Cardiomyopathy is the leading cause of premature death in FRDA. FRDA cardiomyopathy is a complex and progressive disease with no cure or treatment to slow its progression. At the cellular level, cardiomyocyte hypertrophy, apoptosis and fibrosis contribute to the cardiac pathology. However, the heart is composed of multiple cell types and several clinical studies have reported the involvement of cardiac non-myocytes such as vascular cells, autonomic neurons, and inflammatory cells in the pathogenesis of FRDA cardiomyopathy. In fact, several of the cardiac pathologies associated with FRDA including cardiomyocyte necrosis, fibrosis, and arrhythmia, could be contributed to by a diseased vasculature and autonomic dysfunction. Here, we review available evidence regarding the current understanding of cellular mechanisms for and the involvement of cardiac non-myocytes in the pathogenesis of FRDA cardiomyopathy.

弗里德赖希共济失调 (FRDA) 是一种遗传性神经肌肉疾病。心肌病是 FRDA 过早死亡的主要原因。FRDA 心肌病是一种复杂的进行性疾病，无法治愈或治疗以减缓其进展。在细胞水平上，心肌细胞肥大、细胞凋亡和纤维化有助于心脏病理学。然而，心脏由多种细胞类型组成，一些临床研究报道了心脏非肌细胞如血管细胞、自主神经元和炎症细胞参与 FRDA 心肌病的发病机制。事实上，与 FRDA 相关的几种心脏疾病，包括心肌细胞坏死、纤维化和心律失常，可能是由血管系统病变和自主神经功能障碍引起的。这里，