Mayer R J, Tipler C, Laszlo L, Arnold J, Lowe J, Landon M
Department of Biochemistry, University of Nottingham Medical School, Queen's Medical Centre, UK.
Biomed Pharmacother. 1994;48(7):282-6. doi: 10.1016/0753-3322(94)90173-2.
A number of the major human and animal neurodegenerative diseases, such as Alzheimer's disease and sheep scrapie, are characterised by deposits of amyloid, arising through incomplete breakdown of membrane proteins. Although our knowledge concerning these diseases is increasing, they remain largely untreatable. Recently, attention has focussed on the mechanisms of production of different types of amyloid and the likely involvement within cells of acid compartments called endosome-lysosomes. These organelles may be 'bioreactor' sites for the unfolding and partial degradation of membrane proteins to generate the amyloid materials. These subsequently become expelled from the cell, or are released from dead cells, and accumulate as pathological entities. Common features of the disease processes give new direction to therapeutic intervention.
许多主要的人类和动物神经退行性疾病，如阿尔茨海默病和羊瘙痒病，其特征是淀粉样蛋白沉积，这是由于膜蛋白不完全分解产生的。尽管我们对这些疾病的了解在不断增加，但它们在很大程度上仍无法治疗。最近，注意力集中在不同类型淀粉样蛋白的产生机制以及称为内体-溶酶体的酸性区室在细胞内可能的参与情况。这些细胞器可能是膜蛋白展开和部分降解以产生淀粉样物质的“生物反应器”场所。这些物质随后被排出细胞，或从死亡细胞中释放出来，并作为病理实体积累。疾病过程的共同特征为治疗干预提供了新方向。
