Weber M, Pullig O, Köhler H
Department of Internal Medicine, University Erlangen-Nürnberg, FRG.
Nephrol Dial Transplant. 1990;5(2):87-93. doi: 10.1093/ndt/5.2.87.
Collagenase-digested basement-membrane preparations from human kidney glomeruli, kidney tubules, lung, choroid plexus, aorta, intestine, and placenta were analysed according to their reactivity to anti-glomerular basement membrane (anti-GBM) antibody-positive Goodpasture sera. Sodium dodecylsulphate polyacryl gel electrophoresis (SDS-PAGE), and immunoblotting were performed after antigen enrichment by passage of the collagenase digests through an anion-exchange column. Reactivity of anti-GBM antibodies with one to three monomers (24, 26 and 28 kD) and two dimers (44 and 50 kD) were demonstrated in basement membrane preparations of kidney glomeruli, kidney tubules, lung, placenta, and aorta. In basement membranes of choroid plexus reactivity with only the 28 kD monomer and the 50 kD dimer were identified. In intestinal basement membrane, reactivity was restricted to the 50 kD dimer. Analysis of the amounts of Goodpasture antigen by inhibition ELISA demonstrated that the highest concentration were in glomerular basement membrane, while the lowest were found in aortic basement membrane. The results indicate that Goodpasture antigens are common to all the basement membranes investigated. The differences in antigen concentration and in reactivity on immunoblotting may indicate different antigen amounts, a heterogeneity of collagen IV within the various basement membranes, or differences in antigen accessibility within the membranes. We conclude that the primary clinical restriction of the anti-GBM disease to lungs and kidneys is not explained by a preservation of the antigen to this basement membrane. Rather, the clinical pattern may be influenced by differences in the molecular composition of the basement membranes as well as by non-immunological mechanisms.
对来自人肾小球、肾小管、肺、脉络丛、主动脉、肠和胎盘的胶原酶消化的基底膜制剂,根据其与抗肾小球基底膜(抗GBM)抗体阳性的Goodpasture血清的反应性进行分析。在胶原酶消化物通过阴离子交换柱进行抗原富集后,进行十二烷基硫酸钠聚丙烯酰胺凝胶电泳(SDS-PAGE)和免疫印迹。在肾小球、肾小管、肺、胎盘和主动脉的基底膜制剂中,证明抗GBM抗体与一至三种单体(24、26和28kD)以及两种二聚体(44和50kD)有反应性。在脉络丛的基底膜中,仅鉴定出与28kD单体和50kD二聚体有反应性。在肠基底膜中,反应性仅限于50kD二聚体。通过抑制ELISA分析Goodpasture抗原的量表明,最高浓度存在于肾小球基底膜中,而最低浓度存在于主动脉基底膜中。结果表明,Goodpasture抗原在所研究的所有基底膜中都存在。抗原浓度和免疫印迹反应性的差异可能表明抗原量不同、各种基底膜内IV型胶原的异质性或膜内抗原可及性的差异。我们得出结论,抗GBM疾病主要临床局限于肺和肾,并非是因为该基底膜保留了抗原。相反,临床模式可能受基底膜分子组成差异以及非免疫机制的影响。