Recent studies have clearly indicated that amyloid is a generic term which includes a number of different substances, all of which have a beta-pleated sheet structure and a characteristic fibrillar appearance in the electron microscope. The most common types are made of immunoglobulin light chain (AL) fragments in the primary and myeloma-associated type, and of the AA protein in the secondary and some familial forms. It seems probable that other localized types are composed of prohormones or other tissue proteins. In systemic amyloidosis, the fibrils seem to be derived from a soluble circulating precursor; immunoglobulin light chains give rise to AL, and the SAA protein, which behaves as an acute phase reactant yields AA, presumably by proteolysis. It is not known whether the disease is due to overproduction or a defect in degradation. Though effective therapeutic agents are still lacking, colchicine has proved useful in preventing or ameliorating the amyloid in Familial Mediterranean Fever and in several experimental forms of the disease. It deserves careful study as a possible therapeutic agent on other types of amyloidosis.
最近的研究清楚地表明,淀粉样蛋白是一个通用术语,包括许多不同的物质,所有这些物质都具有β-折叠结构,并且在电子显微镜下具有特征性的纤维状外观。最常见的类型在原发性和骨髓瘤相关类型中由免疫球蛋白轻链(AL)片段组成,在继发性和一些家族性形式中由AA蛋白组成。其他局部类型似乎可能由激素原或其他组织蛋白组成。在系统性淀粉样变性中,纤维似乎来源于可溶性循环前体;免疫球蛋白轻链产生AL,而作为急性期反应物的SAA蛋白可能通过蛋白水解产生AA。尚不清楚该疾病是由于产生过多还是降解缺陷所致。尽管仍然缺乏有效的治疗药物,但秋水仙碱已被证明在预防或改善家族性地中海热和几种该疾病的实验形式中的淀粉样变性方面是有用的。作为治疗其他类型淀粉样变性的可能药物,它值得仔细研究。