Ferrario Franco, Rastaldi Maria Pia
Renal Immunopathology Center, S. Carlo Borromeo Hospital and Nuova Nefrologia Research Association, Milan, Italy.
J Nephrol. 2006 Mar-Apr;19(2):123-5.
The term amyloidosis refers to the deposition of an amorphous substance defined by the presence of a fibrillar structure by electron microscopy and a characteristic beta-pleated sheet structure by x-ray diffraction. The existence of this group of diseases has been recognized for centuries, but major advances in the elucidation of the nature of the deposits have been achieved in the past few years. It is now recognized that the beta-sheet configuration responsible for the general appearance of amyloid deposits is common to a large array of proteins with a different origin. This tertiary structure is responsible for the characteristic tinctorial and optical properties evident by Congo red staining. The material that accumulates in the extracellular compartment progressively destroys the involved organ. The accumulation of amyloid deposits may complicate the course of a wide variety of diseases. Classifications of amyloid disorders reflect some of the current concepts concerning the protein composition of amyloid fibrils in different diseases.
术语淀粉样变性指的是一种无定形物质的沉积，这种物质通过电子显微镜观察具有纤维状结构，通过X射线衍射具有特征性的β折叠片层结构。这类疾病的存在已被认识数百年，但在过去几年里，在阐明沉积物性质方面取得了重大进展。现在人们认识到，导致淀粉样沉积物总体外观的β折叠结构在大量来源不同的蛋白质中很常见。这种三级结构导致了刚果红染色所显示的特征性染色和光学性质。在细胞外间隙积累的物质会逐渐破坏受累器官。淀粉样沉积物的积累可能会使多种疾病的病程复杂化。淀粉样变性疾病的分类反映了当前关于不同疾病中淀粉样纤维蛋白组成的一些概念。
