Bergijk E C, Munaut C, Baelde J J, Prins F, Foidart J M, Hoedemaeker P J, Bruijn J A
Department of Pathology, University of Leiden, The Netherlands.
Am J Pathol. 1992 May;140(5):1147-56.
The development of glomerulosclerosis was studied in murine chronic graft-versus-host disease (GvHD), which is a model for human systemic lupus erythematosus. The authors investigated the distribution patterns of six components of the extracellular matrix (ECM), i.e., laminin, fibronectin, collagen types I, III, IV, and VI during the course of the disease. All of these ECM components except collagen type I were found in the glomeruli of normal mice, where all of them were intrinsic constituents of the mesangium. Laminin, fibronectin, and collagen type IV were also found in the glomerular capillary walls. Starting 6 weeks after the induction of GvHD and continuing at week 8, the onset of an expansion of the mesangial matrix was observed. At the same time, the amounts of laminin, fibronectin, and collagen types IV and VI increased. Ten weeks after the onset of the disease, glomerulosclerosis developed. Traces of the interstitial collagen type I were found in sclerotic glomeruli. The levels of four ECM components, i.e., collagens III, IV, VI, and laminin were markedly decreased in the sclerotic glomeruli as compared with week 8. In contrast, the amount of fibronectin in the sclerotic glomeruli increased dramatically. Immunoelectron microscopic examination showed fibronectin in the sclerotic lesions, in contrast to laminin, collagen type I, and collagen type IV. It is concluded that the sclerotic lesions in murine chronic GvHD contain fibronectin. The small amounts of the ECM components laminin, as well as collagens III, IV, and VI in the sclerotic glomeruli in GvHD, might represent remnants of mesangial material and collapsed capillary walls. These components are probably replaced by increased production and/or accumulation of collagen type I and fibronectin.
在小鼠慢性移植物抗宿主病(GvHD)中研究了肾小球硬化的发展情况,该疾病是人类系统性红斑狼疮的一种模型。作者调查了细胞外基质(ECM)六种成分,即层粘连蛋白、纤连蛋白、I型、III型、IV型和VI型胶原蛋白在疾病过程中的分布模式。除I型胶原蛋白外,所有这些ECM成分在正常小鼠的肾小球中均有发现,它们都是系膜的固有成分。层粘连蛋白、纤连蛋白和IV型胶原蛋白也存在于肾小球毛细血管壁中。在GvHD诱导后6周开始并持续到第8周,观察到系膜基质开始扩张。与此同时,层粘连蛋白、纤连蛋白、IV型和VI型胶原蛋白的含量增加。疾病发作10周后,出现肾小球硬化。在硬化的肾小球中发现了少量的间质I型胶原蛋白。与第8周相比,硬化肾小球中四种ECM成分,即III型、IV型、VI型胶原蛋白和层粘连蛋白的水平明显降低。相比之下,硬化肾小球中纤连蛋白的含量急剧增加。免疫电子显微镜检查显示,与层粘连蛋白、I型胶原蛋白和IV型胶原蛋白不同,硬化病变中有纤连蛋白。得出的结论是,小鼠慢性GvHD中的硬化病变含有纤连蛋白。GvHD硬化肾小球中层粘连蛋白以及III型、IV型和VI型胶原蛋白等少量ECM成分可能代表系膜物质和塌陷毛细血管壁的残余物。这些成分可能被I型胶原蛋白和纤连蛋白产量增加和/或积累所取代。