论文部分内容阅读
肾小球基膜(GBM)是肾小球滤过蛋白的主要屏障,它不但可作为免疫复合物的沉积处,它的某些成份还可作为抗原引起相应的抗体,产生抗 GBM 抗体疾病。现认为 Goodpasture 综合征(肺肾综合征)与抗 GBM 抗体疾病不完全相同。Goodpasture 综合征可存在抗 GBM 抗体,也可不存在。有些存在抗GBM 抗体的肾炎又可不表现为 Goodpasture 综合征。GBM 是由蛋白质和葡萄糖氨基聚糖组成的结构。胶原是主要蛋白质成份,并主要是以前胶原形式存在的Ⅳ型胶原。Ⅳ型胶原上的羟赖氨酸-半乳糖-葡萄糖复合物可作为抗原。实验证明它可以与抗 GBM抗体疾病患者血清中或肾组织中的抗体起反应。用抗Ⅳ型胶原的抗体注射于兔子可引起肺出血和肾小球炎症。在19例 Goodpasture 综合征患者的血液中测得抗Ⅳ型前胶原抗体者有15例。在 GBM 中除胶原蛋白
Glomerular basement membrane (GBM) is the main barrier of glomerular filtration protein, it not only can be deposited as immune complexes, but also some of its components can also cause anti-GBM antibodies, resulting in anti-GBM antibody disease. Now that Goodpasture syndrome (lung and kidney syndrome) and anti-GBM antibody disease is not exactly the same. Goodpasture syndrome may exist anti-GBM antibodies, may also be absent. Some nephritis in the presence of anti-GBM antibodies may not show up as Goodpasture’s syndrome. GBM is a structure composed of proteins and glycosaminoglycans. Collagen is a major protein component and is predominantly type IV collagen in the form of previous collagen. Hydroxylysine-galactose-glucose complexes on type IV collagen are useful as antigens. Experiments show that it can react with anti-GBM antibodies in serum or in kidney tissue. Injection of anti-type IV collagen into rabbits can cause pulmonary hemorrhage and glomerular inflammation. Fifteen patients had anti-type IV procollagen antibodies in the blood of 19 patients with Goodpasture’s syndrome. In addition to collagen in the GBM