Fundamental studies on the biochemical mechanism of chemical chaperoning of glucocerebrosidase

葡萄糖脑苷脂酶化学伴侣生化机制的基础研究

• 批准号: 480335-2015
• 负责人: Bennet, Andrew
• 金额: $ 15.3万
• 依托单位: Simon Fraser University
• 依托单位国家: 加拿大
• 项目类别: Collaborative Research and Development Grants
• 财政年份: 2016
• 资助国家: 加拿大
• 起止时间: 2016-01-01 至 2017-12-31
• 项目状态: 已结题
• 来源: https://www.nserc-crsng.gc.ca/ase-oro/Details-Detailles_eng.asp?id=599963
• 关键词: Fundamental; studies; biochemical; mechanism; chemical

Parkinson's disease is a worldwide health problem that has been linked to deficiencies in an enzyme called glucocerebrosidase. This enzyme plays a critical role in the body by breaking down a particular large molecule into two smaller, more easily metabolized components, a sugar (glucose) and a fat (ceramide). Without a properly functioning glucocerebrosidase, these large molecules are not degraded in a normal manner. Genetic mutations associated with glucocerebrosidase often result in an unstable enzyme structure that is easily damaged in the intracellular environment. In the case of Parkinson's disease, loss of function of this enzyme may contribute to disease progression.

帕金森病是一个世界性的健康问题，与一种名为葡萄糖脑苷脂酶的酶缺乏有关。这种酶在体内起着关键作用，它将一种特定的大分子分解成两种更小、更容易代谢的成分，一种糖（葡萄糖）和一种脂肪（神经酰胺）。如果没有功能正常的葡萄糖脑苷脂酶，这些大分子就不能以正常的方式降解。与葡糖脑苷脂酶相关的基因突变通常导致不稳定的酶结构，其在细胞内环境中容易受损。在帕金森病的情况下，这种酶的功能丧失可能会导致疾病进展。