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糖原代谢与结构:综述。

Glycogen metabolism and structure: A review.

机构信息

School of Medicine, Shanghai University, Shanghai 200444, China.

Centre for Nutrition and Food Sciences, Queensland Alliance for Agriculture and Food Innovation, The University of Queensland, Brisbane, Queensland 4072, Australia; Centre for Animal Science, Queensland Alliance for Agriculture and Food Innovation, The University of Queensland, Brisbane, Queensland 4072, Australia.

出版信息

Carbohydr Polym. 2024 Dec 15;346:122631. doi: 10.1016/j.carbpol.2024.122631. Epub 2024 Aug 17.

Abstract

Glycogen is a glucose polymer that plays a crucial role in glucose homeostasis by functioning as a short-term energy storage reservoir in animals and bacteria. Abnormalities in its metabolism and structure can cause several problems, including diabetes, glycogen storage diseases (GSDs) and muscular disorders. Defects in the enzymes involved in glycogen synthesis or breakdown, resulting in either excessive accumulation or insufficient availability of glycogen in cells seem to account for the most common pathogenesis. This review discusses glycogen metabolism and structure, including molecular architecture, branching dynamics, and the role of associated components within the granules. The review also discusses GSD type XV and Lafora disease, illustrating the broader implications of aberrant glycogen metabolism and structure. These conditions also impart information on important regulatory mechanisms of glycogen, which hint at potential therapeutic targets. Knowledge gaps and potential future research directions are identified.

摘要

糖原是一种葡萄糖聚合物,在动物和细菌中作为短期能量储存库,对葡萄糖稳态起着至关重要的作用。其代谢和结构的异常可导致多种问题,包括糖尿病、糖原贮积病(GSD)和肌肉疾病。参与糖原合成或分解的酶的缺陷,导致细胞内糖原的过度积累或不足,似乎是最常见的发病机制。本文综述了糖原的代谢和结构,包括分子结构、分支动力学以及颗粒内相关成分的作用。本文还讨论了 GSD 类型 XV 和 Lafora 病,说明了异常糖原代谢和结构的更广泛影响。这些疾病还提供了关于糖原重要调节机制的信息,提示了潜在的治疗靶点。本文确定了知识空白和潜在的未来研究方向。

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