Summary
Skin fibroblasts and tumor cells were cultured from four patients with peripheral von Recklinghausen's neurofibromatosis (NF). The cell type enriched in culture from the tumors carried the fibroblastic Thy 1.1. cell surface antigen and produced fibronectin, like fibroblasts from skin of NF-patients or from control persons. In electron micrographs the NF tumor and NF skin cells were similar to the control skin fibroblasts; elongated in shape, contained tubular mitochondria, variable amounts of granular endoplasmic reticulum, numerous lysosomal inclusion bodies and collections of 5 nm filaments. Trypsinized cells were fractionated with centrifugation in a Percoll density gradient. All cell lines produced only one sharp band of viable cells at the buoyant density of 1.03.
Compared with the NF skin or control skin fibroblasts the NF tumor cells, however, produced a less well organized peri-and extracellular matrix estimated from fibronectin fluorescence. The nuclear sizes were measured from photographs of the cultures. The nuclei of all four tumor cell lines were larger than those of the skin fibroblasts of the corresponding patients. Neurofibromatosis tumor cells thus resemble skin fibroblasts in their density and in some ultrastructural properties but are different in their growth pattern and synthetic functions.
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Financially supported by grants (to J.P.) from the Research and Scientific Foundation of Lääke Oy Ltd. and from the Emil and Blida Maunula Foundation, and by institutional grants from the Turku University Foundation and the Sigrid Jusélius Foundation
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Peltonen, J., Aho, H., Rinne, U.K. et al. Neurofibromatosis tumor and skin cells in culture. Acta Neuropathol 61, 275–282 (1983). https://doi.org/10.1007/BF00691998
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DOI: https://doi.org/10.1007/BF00691998