Characterization of cultured neurofibroma cells derived from von Recklinghausen's disease

We have examined the cytoplasmic distribution of actin and fibronectin in cultured neurofibroma cells (NF cells) derived from a patient with von Recklinghausen's disease by using phase contrast and indirect immunofluorescence microscopy. NF cells were larger in size and more dendritic in shape compared to normal human dermal fibroblasts. NF cells also showed abundant granular staining of actin and a decrease in the linear staining pattern of fibronectin. Furthermore, employing a colony-formation assay on the top of an agar-gel in the presence of fibroblast growth factor (FGF), normal fibroblasts showed a significant number of colonies, whereas NF cells did not demonstrate colony formation even after addition of FGF. These findings suggests that NF cells from patients with von Recklinghausen's disease may have different characteristics when compared with normal fibroblasts, and that NF cells are similar to transformed cells with regard to their actin and fibronectin distribution.