Mitochondria play a prominent role in cardiac energy metabolism, and their function is critically dependent on the integrity of mitochondrial membranes. Disorders characterized by mitochondrial dysfunction are commonly associated with cardiac disease. The mitochondrial phospholipid cardiolipin directly interacts with a number of essential protein complexes in the mitochondrial membranes including the respiratory chain, mitochondrial metabolite carriers, and proteins critical for mitochondrial morphology. Barth syndrome is an X-linked disorder caused by an inherited defect in the biogenesis of the mitochondrial phospholipid cardiolipin. How cardiolipin deficiency impacts on mitochondrial function and how mitochondrial dysfunction causes cardiomyopathy has been intensively studied in cellular and animal models of Barth syndrome. These findings may also have implications for the molecular mechanisms underlying other inherited disorders associated with defects in cardiolipin, such as Sengers syndrome and dilated cardiomyopathy with ataxia (DCMA).

中文翻译：

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Barth综合征和其他遗传性心肌病中的心磷脂重塑。

线粒体在心脏能量代谢中起着重要作用，其功能关键取决于线粒体膜的完整性。以线粒体功能障碍为特征的疾病通常与心脏病有关。线粒体磷脂心磷脂直接与线粒体膜中的许多必需蛋白质复合物相互作用，包括呼吸链，线粒体代谢物载体和对线粒体形态至关重要的蛋白质。Barth综合征是由线粒体磷脂心磷脂的生物发生中的遗传缺陷引起的X连锁疾病。在Barth综合征的细胞和动物模型中，心磷脂缺乏如何影响线粒体功能以及线粒体功能障碍如何引起心肌病。