Pietromonaco S, Kerjaschki D, Binder S, Ullrich R, Farquhar M G
Department of Cell Biology, Yale University School of Medicine, New Haven, CT 06510.
Proc Natl Acad Sci U S A. 1990 Mar;87(5):1811-5. doi: 10.1073/pnas.87.5.1811.
Heymann nephritis is an experimental autoimmune disease in rats that is characterized by accumulation of immune deposits (IDs) in kidney glomeruli. The disease is initiated by the binding of circulating antibodies to a membrane glycoprotein, gp330, which is a resident protein of clathrin-coated pits on glomerular epithelial cells (podocytes). We have defined a domain representing about 10% of gp330 that appears to be responsible for the formation of stable glomerular IDs. A cDNA clone (clone 14) was isolated from a rat kidney cDNA expression library by screening with IgG eluted from glomeruli of rats in early stages (3 days) of passive Heymann nephritis. The clone 14 cDNA contains an open reading frame encoding the C-terminal 319 amino acids of gp330. The predicted amino acid sequence contains four internal repeats of 11 amino acids, which are also found in the putative ligand-binding region of carbohydrate-binding lectin-like receptors. An antibody raised against the clone 14 fusion protein recognized gp330 by immunoblotting and induced formation of subepithelial IDs typical of passive Heymann nephritis when injected into normal rats. When the clone 14 fusion protein was used to immunize rats, subepithelial IDs of active Heymann nephritis were found after 12 weeks. No IDs were formed by active or passive immunization of rats with fusion proteins derived from other regions of gp330. These results demonstrate that clone 14 encodes a region of gp330 responsible for antibody binding and ID formation in vivo.
海曼肾炎是一种大鼠实验性自身免疫性疾病,其特征是免疫沉积物(IDs)在肾小球中积聚。该疾病由循环抗体与一种膜糖蛋白gp330结合引发,gp330是肾小球上皮细胞(足细胞)上网格蛋白包被小窝的驻留蛋白。我们确定了一个约占gp330 10%的结构域,该结构域似乎负责稳定肾小球IDs的形成。通过用从被动海曼肾炎早期(3天)大鼠肾小球洗脱的IgG进行筛选,从大鼠肾脏cDNA表达文库中分离出一个cDNA克隆(克隆14)。克隆14 cDNA包含一个开放阅读框,编码gp330的C末端319个氨基酸。预测的氨基酸序列包含四个11个氨基酸的内部重复序列,这些重复序列也存在于碳水化合物结合凝集素样受体的假定配体结合区域。针对克隆14融合蛋白产生的抗体通过免疫印迹识别gp330,并在注入正常大鼠时诱导形成被动海曼肾炎典型的上皮下IDs。当用克隆14融合蛋白免疫大鼠时,12周后发现了主动海曼肾炎的上皮下IDs。用gp330其他区域衍生的融合蛋白对大鼠进行主动或被动免疫均未形成IDs。这些结果表明,克隆14编码了gp330中负责体内抗体结合和IDs形成的区域。