The glycogen storage diseases are a group of inherited metabolic disorders that are characterized by specific enzymatic defects involving the synthesis or degradation of glycogen. Each disorder presents with a set of symptoms that are due to the underlying enzyme deficiency and the particular tissues that are affected. Autophagy is a process by which cells degrade and recycle unneeded or damaged intracellular components such as lipids, glycogen, and damaged mitochondria. Recent studies showed that several of the glycogen storage disorders have abnormal autophagy which can disturb normal cellular metabolism and/or mitochondrial function. Here, we provide a clinical overview of the glycogen storage disorders, a brief description of autophagy, and the known links between specific glycogen storage disorders and autophagy.

中文翻译：

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自噬与糖原代谢紊乱之间的联系 - 发病机制和可能治疗的观点。

糖原贮积病是一组遗传性代谢紊乱，其特征是涉及糖原合成或降解的特定酶缺陷。每种疾病都会出现一系列症状，这些症状是由于潜在的酶缺乏和受影响的特定组织造成的。自噬是细胞降解和回收不需要或受损的细胞内成分（例如脂质、糖原和受损的线粒体）的过程。最近的研究表明，一些糖原储存障碍具有异常的自噬，可以扰乱正常的细胞代谢和/或线粒体功能。在这里，我们提供糖原贮积症的临床概述、自噬的简要描述以及特定糖原贮积症和自噬之间的已知联系。