Lundstrom M, Orlando R A, Saedi M S, Woodward L, Kurihara H, Farquhar M G
Division of Cellular and Molecular Medicine, University of California, San Diego, La Jolla 92093-0651.
Am J Pathol. 1993 Nov;143(5):1423-35.
Heymann nephritis in the rat is the most widely used model of human membranous glomerulonephritis. Glycoprotein (gp)330, a large (M(r) > 550,000) membrane-associated glycoprotein, has been identified as the main antigen in this autoimmune disease. Studies of gp330 and receptor-associated protein (RAP), its 44-kd subunit, have been restricted largely to rat kidney, as no stable cultured cell line has been available that expresses gp330. We have recently identified a rat yolk sac carcinoma cell line (L2) that expresses both gp330 and RAP. In this report, we have carried out detailed morphological, immunocytochemical, and biochemical studies characterizing the biosynthesis and localization of gp330 and RAP in the L2 rat yolk sac cell line. At the electron microscope level, the L2 cells are seen to be attached by cell junctions, and their predominant morphological features include extensive networks of rough endoplasmic reticulum (ER) and numerous clathrin-coated pits found on the cell membrane. By immunocytochemistry, gp330 was localized primarily to clathrin-coated pits at the cell surface, whereas RAP was localized predominantly to the lumen of the rough ER. Pulse-chase experiments indicated that gp330 spends a prolonged time maturing in the ER of L2 cells, as transport of gp330 to the Golgi complex (based on acquisition of endoglycosidase H resistance) is slow (t1/2 = 90 to 120 minutes). Gp330 reached the L2 cell surface beginning at 2 hours after synthesis, where it could be detected by cell surface immunoprecipitation. RAP was found to be an N-linked glycoprotein, and it remained endoglycosidase H-sensitive up to 4 hours after synthesis. Co-precipitation and co-sedimentation experiments demonstrated that gp330 and RAP form a large heterodimer (M(r) approximately 669,000) immediately after biosynthesis and are further assembled into a large hetero-oligomer in the ER. These findings demonstrate that the localization and the kinetics of assembly of gp330 and RAP into the Heymann nephritis antigenic complex are similar in both L2 cells and rat kidney. They also provide new information on the intracellular processing of these two molecules and their delivery to the cell surface. Thus, the L2 cell system should facilitate further characterization of the functions and interactions of gp330 and RAP, which may shed light on the cellular and molecular mechanisms of Heymann nephritis.
大鼠的海曼肾炎是人类膜性肾小球肾炎中应用最为广泛的模型。糖蛋白(gp)330是一种大型(分子量>550,000)的膜相关糖蛋白,已被确定为这种自身免疫性疾病中的主要抗原。由于一直没有可稳定表达gp330的培养细胞系,对gp330及其44-kd亚基受体相关蛋白(RAP)的研究主要局限于大鼠肾脏。我们最近鉴定出一种大鼠卵黄囊癌细胞系(L2),它能同时表达gp330和RAP。在本报告中,我们进行了详细的形态学、免疫细胞化学和生物化学研究,以表征gp330和RAP在L2大鼠卵黄囊细胞系中的生物合成及定位。在电子显微镜水平上,可以看到L2细胞通过细胞连接相连,其主要形态特征包括广泛的粗面内质网(ER)网络以及细胞膜上大量的网格蛋白包被小窝。通过免疫细胞化学方法,gp330主要定位于细胞表面的网格蛋白包被小窝,而RAP主要定位于粗面内质网腔。脉冲追踪实验表明,gp330在L2细胞的内质网中成熟所需时间较长,因为gp330向高尔基体复合体的转运(基于获得内切糖苷酶H抗性)较慢(半衰期=90至120分钟)。gp330在合成后2小时开始到达L2细胞表面,在那里可以通过细胞表面免疫沉淀检测到。发现RAP是一种N-连接糖蛋白,在合成后长达4小时内对内切糖苷酶H仍敏感。共沉淀和共沉降实验表明,gp330和RAP在生物合成后立即形成一个大型异二聚体(分子量约669,000),并在内质网中进一步组装成一个大型异寡聚体。这些发现表明,gp330和RAP在L2细胞和大鼠肾脏中定位到海曼肾炎抗原复合物的过程及组装动力学相似。它们还提供了关于这两种分子细胞内加工过程及其向细胞表面转运的新信息。因此,L2细胞系统应有助于进一步表征gp330和RAP的功能及相互作用,这可能会揭示海曼肾炎细胞和分子机制。