Kivelä T, Virtanen I, Marcus D M, O'Brien J M, Carpenter J L, Brauner E, Tarkkanen A, Albert D M
Department of Ophthalmology, University of Helsinki, Finland.
Am J Pathol. 1991 May;138(5):1135-48.
Antigenic properties of a murine transgenic model for hereditary retinoblastoma, induced by a chimeric gene coding for Simian virus 40 large T antigen, an oncogene that inactivates the retinoblastoma susceptibility gene product, were studied by immunohistochemistry. All transgenic mice develop bilateral intraocular retinal tumors in the inner nuclear layer with Homer Wright-like rosettes, and one quarter develop midbrain tumors resembling trilateral retinoblastoma. Cell lines TE-1 and TM-1 were established from intraocular and metastatic tumors, respectively. Intraocular tumors reacted with antibodies to neuron-specific enolase and synaptophysin, while vimentin, glial fibrillary acidic, and S-100 proteins were detected only in reactive glia derived from adjacent retina. The midbrain tumors showed weak reactivity to synaptophysin, and they blended with reactive astrocytes positive for glial markers. The tumors were negative for cytokeratins. Finally both derived cell lines expressed synaptophysin and individual neurofilament triplet proteins in immunofluorescence and Western blotting, supporting their essentially neuronal nature. The antigenic profile resembles human retinoblastoma, but differences in morphology and antigen distribution suggest a more close relationship to neurons of the inner nuclear layer than to photoreceptor cells.
通过免疫组织化学研究了一种遗传性视网膜母细胞瘤小鼠转基因模型的抗原特性，该模型由编码猿猴病毒40大T抗原的嵌合基因诱导产生，该癌基因可使视网膜母细胞瘤易感基因产物失活。所有转基因小鼠在内核层均发生双侧眼内视网膜肿瘤，并伴有霍默·赖特样菊形团，四分之一的小鼠发生类似三边性视网膜母细胞瘤的中脑肿瘤。分别从眼内肿瘤和转移性肿瘤建立了细胞系TE-1和TM-1。眼内肿瘤与神经元特异性烯醇化酶和突触素抗体发生反应，而波形蛋白、胶质纤维酸性蛋白和S-100蛋白仅在相邻视网膜来源的反应性胶质细胞中检测到。中脑肿瘤对突触素显示出弱反应性，并且它们与胶质标记物阳性的反应性星形胶质细胞混合。肿瘤细胞角蛋白呈阴性。最后，两种衍生细胞系在免疫荧光和蛋白质印迹中均表达突触素和单个神经丝三联体蛋白，支持它们本质上的神经元性质。抗原谱类似于人类视网膜母细胞瘤，但形态和抗原分布的差异表明其与内核层神经元的关系比与光感受器细胞的关系更密切。
