Trojanowski J Q, Friedman H S, Burger P C, Bigner D D
Am J Pathol. 1987 Feb;126(2):358-63.
The majority of cells in a rapidly dividing human medulloblastoma cell line (D283 MED) are shown to express the two high-molecular-weight human neurofilament (NF) subunits, whereas a minority express the low-molecular-weight NF subunit. These three polypeptides are integral subunits of the intermediate filaments (IFs) found in normal neurons. Other cell type-specific IF proteins (keratin, desmin, and glial filament polypeptides) are not present in D283 MED cells. Further, the immunocytochemical, immunochemical and ultrastructural data suggest that the neurofilaments in these cells are abnormal, possibly because of a paucity of the low-molecular-weight NF subunit. This is the first human cell line derived from a central nervous system tumor that is capable of expressing all three NF triplet proteins. It is a unique model system for studies of normal and abnormal human NF metabolism as well as for probing the cell biology of medulloblastomas.
在快速分裂的人髓母细胞瘤细胞系（D283 MED）中，大多数细胞显示表达两种高分子量的人神经丝（NF）亚基，而少数细胞表达低分子量的NF亚基。这三种多肽是正常神经元中中间丝（IFs）的组成亚基。其他细胞类型特异性的IF蛋白（角蛋白、结蛋白和胶质纤维多肽）在D283 MED细胞中不存在。此外，免疫细胞化学、免疫化学和超微结构数据表明，这些细胞中的神经丝是异常的，可能是由于低分子量NF亚基的缺乏。这是第一个源自中枢神经系统肿瘤且能够表达所有三种NF三联体蛋白的人细胞系。它是研究正常和异常人NF代谢以及探究髓母细胞瘤细胞生物学的独特模型系统。
