Kitano Y, Okamoto E, Saito K, Okano Y
Department of Dermatology, Hyogo College of Medicine, Japan.
J Dermatol Sci. 1992 May;3(3):137-44. doi: 10.1016/0923-1811(92)90027-9.
Neurofibromatosis type 1 (NF1) is a common autosomal dominant disorder characterized by abnormalities affecting multiple tissues derived from the neural crest. The peripheral neurofibromas are numerous and sometimes reach several hundred in number. In this study, the possible involvement of several growth factors in neurofibroma growth was investigated in vitro. When explants of neurofibroma tissue were cultured, macrophage-like cells with pseudopodia migrated out first, and later took on a slender fusiform shape. These cells contained S-100 protein and were identified as Schwann cells. They did not proliferate under standard culture conditions. Nerve growth factor (NGF) was helpful in maintaining the differentiated phenotype of Schwann cells, but did not stimulate their proliferation. Immunohistochemical staining for type IV collagen revealed that some large flattened polygonal cells had a mesh of type IV collagen on the surface. These cells were perineurial cells. The proliferation of cells derived from neurofibroma was stimulated by basic fibroblast growth factor (bFGF), epidermal growth factor (EGF), and transforming growth factor alpha (TGF-alpha). In comparison with skin fibroblasts, the cells derived from neurofibroma responded to these growth factors at considerably lower concentrations. Stimulation by EGF at physiological concentrations indicated the possible involvement of EGF in the development of neurofibromas.
1型神经纤维瘤病（NF1）是一种常见的常染色体显性疾病，其特征是影响源自神经嵴的多个组织的异常。周围神经纤维瘤数量众多，有时可达数百个。在本研究中，在体外研究了几种生长因子在神经纤维瘤生长中的可能作用。当培养神经纤维瘤组织的外植体时，带有伪足的巨噬细胞样细胞首先迁移出来，随后呈现细长的梭形。这些细胞含有S-100蛋白，被鉴定为施万细胞。在标准培养条件下它们不增殖。神经生长因子（NGF）有助于维持施万细胞的分化表型，但不刺激其增殖。IV型胶原的免疫组织化学染色显示，一些大的扁平多边形细胞表面有IV型胶原网。这些细胞是神经束膜细胞。碱性成纤维细胞生长因子（bFGF）、表皮生长因子（EGF）和转化生长因子α（TGF-α）刺激神经纤维瘤来源的细胞增殖。与皮肤成纤维细胞相比，神经纤维瘤来源的细胞对这些生长因子的反应浓度要低得多。生理浓度的EGF刺激表明EGF可能参与神经纤维瘤的发生。
