Fleischmajer R, Jacobs L, Schwartz E, Sakai L Y
Department of Dermatology, Mount Sinai School of Medicine, New York.
Lab Invest. 1991 Jun;64(6):791-8.
Extracellular microfibrils, about 10 nm thick with a hollow core have been found in most organs as free bundles or in association with elastic fibrils. Histochemistry of the dermis of 4 patients with localized and 6 with systemic scleroderma revealed numerous fine elastic fibrils in areas of fibrosis. Immunofluorescence and immunoelectron microscopy were performed with antibodies against fibrillin and amorphous elastin. The lower dermis revealed an increase in 10-nm microfibrils interspersed between collagen fibrils. These microfibrils stained for fibrillin but not for amorphous elastin. Fibrosis in localized and systemic scleroderma involves the deposition of collagen fibrils and microfibrils.
已在大多数器官中发现细胞外微原纤维，其厚度约为10纳米，有一个中空的核心，呈游离束状或与弹性纤维相关联。对4例局限性硬皮病患者和6例系统性硬皮病患者的真皮进行组织化学检查，发现在纤维化区域有许多细小的弹性纤维。用抗原纤蛋白和无定形弹性蛋白的抗体进行免疫荧光和免疫电子显微镜检查。真皮下层显示10纳米的微原纤维在胶原纤维之间散布增加。这些微原纤维对原纤蛋白呈阳性染色，但对无定形弹性蛋白呈阴性染色。局限性和系统性硬皮病中的纤维化涉及胶原纤维和微原纤维的沉积。
