Type VI collagen. In situ hybridizations and immunohistochemistry reveal abundant mRNA and protein levels in human neurofibroma, schwannoma and normal peripheral nerve tissues.
Type VI collagen. In situ hybridizations and immunohistochemistry reveal abundant mRNA and protein levels in human neurofibroma, schwannoma and normal peripheral nerve tissues.
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发表时间：
1990-04
期刊：
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通讯作者：
Juha Peltonen;S. Jaakkola;L. Hsiao;R. Timpl;Mon-Li Chu;Jouni Uitto
中科院分区：
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作者：
Juha Peltonen;S. Jaakkola;L. Hsiao;R. Timpl;Mon-Li Chu;Jouni Uitto
皮肤神经纤维瘤含有广泛的细胞外基质，由胶原和非胶原大分子组成。在这项研究中，VI型胶原基因在皮肤神经纤维瘤的表达进行了研究，结合原位杂交和免疫组化。原位杂交与32 P-标记的人VI型胶原特异性cDNA显示，神经纤维瘤内的大多数细胞表达α 2（VI）胶原链的基因。表达明显可检测水平的α 2（VI）胶原mRNA的细胞数量显著高于活跃表达前α 1（I）或前α 1（III）胶原基因的细胞数量。神经纤维瘤内VI型胶原蛋白表位的存在也证明了特异性多克隆抗体的免疫染色。VI型胶原基因在神经组织中的表达通过由雪旺细胞组成的良性神经鞘瘤组织标本的免疫染色进一步检查。结果表明VI型胶原表位与肿瘤性雪旺细胞密切相关。VI型胶原蛋白表位在正常人外周神经内的免疫定位显示神经束膜细胞和雪旺细胞的细胞周染色，表明这些细胞类型的VI型胶原蛋白的合成。这些结果表明，VI型胶原基因的表达是活跃的神经源性组织，和VI型胶原可能是神经结缔组织中的细胞外基质的主要成分。
Cutaneous neurofibromas contain an extensive extracellular matrix composed of collagenous and non-collagenous macromolecules. In this study, the expression of type VI collagen genes in cutaneous neurofibromas was examined by a combination of in situ hybridizations and immunohistochemistry. In situ hybridizations with a 32P-labeled human type VI collagen-specific cDNA revealed that the majority of cells within neurofibromas expressed the gene for alpha 2(VI) collagen chain. The number of cells expressing clearly detectable levels of alpha 2(VI) collagen mRNA was considerably higher than that of cells actively expressing the pro alpha 1(I) or pro alpha 1(III) collagen genes. The presence of type VI collagen epitopes within the neurofibromas was also demonstrated by immunostaining with specific polyclonal antibodies. The expression of type VI collagen genes in neural tissues was further examined by immunostaining of a benign schwannoma tissue specimen consisting of Schwann cells. The results indicated close association of type VI collagen epitopes with the neoplastic Schwann cells. Immunolocalization of type VI collagen epitopes within normal human peripheral nerve revealed pericellular staining of perineurial cells and Schwann cells, suggesting synthesis of type VI collagen by these cell types. These results suggest that the expression of type VI collagen gene is active in nerve-derived tissues, and that type VI collagen may be a major component of the extracellular matrix in neural connective tissues.