拉福拉病
糖原
糖原合酶
糖原脱支酶
糖原贮积病
生物
糖原分支酶
碳水化合物代谢
生物化学
酶
磷酸酶
作者
Galex K.S. Neoh,Xinle Tan,Si Chen,Eugeni Roura,Xin Dong,Robert G. Gilbert
标识
DOI:10.1016/j.carbpol.2024.122631
摘要
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.
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