Kisilevsky R
Department of Pathology, Queen's University, Kingston, Ontario, Canada.
Mod Pathol. 1991 Jul;4(4):514-8.
Amyloidosis is not one entity but a grouping of disorders in all of which there is a tissue protein deposit with similar morphological, structural, and staining properties despite involving fundamentally different proteins or peptides. Each protein is involved in its own specific pathogenetic mechanism. These mechanisms determine, in a manner as yet unknown, which protein will be present in an amyloid deposit. The role of proteolysis in the pathogenesis is questioned. Proteolysis may well be a postfibrillogenic event. Local tissue factors, such as the basement membrane form of heparan sulfate proteoglycan, in addition to perhaps other basement membrane components, may play an important role in the genesis of amyloid. Differences, common themes, and various unanswered questions related to the pathogenesis of the amyloids are presented as are practical diagnostic considerations.
淀粉样变性并非单一疾病,而是一组病症,在所有这些病症中,尽管涉及根本不同的蛋白质或肽,但都存在具有相似形态、结构和染色特性的组织蛋白沉积物。每种蛋白质都参与其自身特定的致病机制。这些机制以一种尚不清楚的方式决定哪种蛋白质会存在于淀粉样沉积物中。蛋白水解在发病机制中的作用受到质疑。蛋白水解很可能是原纤维形成后的事件。局部组织因子,如硫酸乙酰肝素蛋白聚糖的基底膜形式,可能还有其他基底膜成分,可能在淀粉样蛋白的形成中起重要作用。文中介绍了与淀粉样变性发病机制相关的差异、共同主题以及各种未解决的问题,还有实际的诊断考量。