Casanova S, Donini U, Zucchelli P, Mazzucco G, Monga G, Linke R P
Laboratorio di Microscopia Elettronica, Ospedale M. Malpighi, Bologna, Italy.
Am J Clin Pathol. 1992 Jun;97(6):787-95. doi: 10.1093/ajcp/97.6.787.
Six patients with glomerulonephritis and glomerular proteinaceous deposits constituted by fibrillar ultrastructures similar to those of amyloid but lacking the Congo red tinctorial affinity characterizing amyloid were studied. Clinically, these patients had proteinuria and hematuria; in addition, three patients had hypertension and one renal failure. Protein deposits in their kidney biopsy sections were evaluated by immunofluorescence, immunoperoxidase, and immunoelectron microscopic (protein A-gold) techniques, using antibodies against IgG, IgA, IgM, C3, C1q, fibrinogen, immunoglobulin kappa and lambda light chains, and against amyloid fibril proteins of different types, including AA, A lambda, A kappa, and AF. By immunofluorescence and immunoperoxidase, in all cases the deposits stained intensely with antibodies against IgG, C3, and kappa and lambda light chains; one case also showed C1q immunoreactivity. By contrast, none stained with antibodies against various amyloid fibril proteins. Immunoelectron microscopic findings corroborated this data, indicating that the nonamyloid fibrillar deposits studied are antigenically distinct from known amyloid deposits and that they contain IgG-derived material.
对6例患有肾小球肾炎且肾小球存在由纤维状超微结构构成的蛋白质沉积物的患者进行了研究,这些纤维状超微结构与淀粉样蛋白相似,但缺乏淀粉样蛋白特有的刚果红染色亲和力。临床上,这些患者有蛋白尿和血尿;此外,3例患者有高血压,1例有肾衰竭。使用针对IgG、IgA、IgM、C3、C1q、纤维蛋白原、免疫球蛋白κ和λ轻链以及不同类型淀粉样纤维蛋白(包括AA、Aλ、Aκ和AF)的抗体,通过免疫荧光、免疫过氧化物酶和免疫电子显微镜(蛋白A-金)技术对其肾活检切片中的蛋白质沉积物进行评估。通过免疫荧光和免疫过氧化物酶检测,在所有病例中,沉积物均被针对IgG、C3以及κ和λ轻链的抗体强烈染色;1例还显示出C1q免疫反应性。相比之下,没有一例被针对各种淀粉样纤维蛋白的抗体染色。免疫电子显微镜检查结果证实了这一数据,表明所研究的非淀粉样纤维状沉积物在抗原性上与已知的淀粉样沉积物不同,并且它们含有IgG衍生物质。