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目的检测神经纤维瘤蛋白在Ⅰ型神经纤维瘤病(type1neurofibromatosis,NF1)脊柱侧凸患者成骨细胞中的表达,观察NF1脊柱侧凸患者成骨细胞的生物学特性。方法先天性脊柱侧凸患者10例,NF1脊柱侧凸患者8例,两组年龄和Cobb角相近。脊柱后路手术取髂骨松质骨,采用植块法进行成骨细胞培养。取第二代成骨细胞分别检测其增殖活性、碱性磷酸酶、Ⅰ型胶原和骨钙素等成骨细胞特异性分化指标,并采用免疫沉淀和Westernblot法检测神经纤维瘤蛋白在两组成骨细胞中的表达。结果NF1脊柱侧凸患者成骨细胞中神经纤维瘤蛋白表达水平明显低于先天性脊柱侧凸患者(灰度积分比值分别为1.05±0.06和2.59±1.40,P=0.002);碱性磷酸酶、Ⅰ型胶原和骨钙素水平均明显低于先天性脊柱侧凸患者(44.69IU/mg和51.38IU/mg,P=0.019;226.34ng/mg和249.93ng/mg,P=0.014;7.41ng/mg和8.87ng/mg,P=0.049)。NF1脊柱侧凸患者成骨细胞显示相对活跃的增殖活性(增殖倍数分别为3.34和2.70,P=0.049)。结论NF1脊柱侧凸患者成骨细胞神经纤维瘤蛋白表达降低的同时,其功能存在明显缺陷。成骨细胞功能缺陷可能是骨骼营养不良性改变和骨密度降低等各种骨骼异常共同的基础。
Objective To detect the expression of neurofibromin in osteoblasts of patients with type1 neurofibromatosis (NF1) scoliosis and to observe the biological characteristics of osteoblasts in patients with NF1 scoliosis. Methods 10 cases of congenital scoliosis patients, 8 cases of NF1 scoliosis patients, two groups of similar age and Cobb angle. Posterior spine surgery to take iliac cancellous bone, osteoblast culture using explant method. The second generation of osteoblasts were used to detect the specific differentiation of osteoblasts, such as proliferation activity, alkaline phosphatase, type I collagen and osteocalcin. Immunoprecipitation and Western blotting were used to detect the expression of neurofibromin in osteoblasts Expression in cells. Results The expression of neurofibromia protein in osteoblasts of NF1 scoliosis patients was significantly lower than that of patients with congenital scoliosis (gray integral score was 1.05 ± 0.06 and 2.59 ± 1.40, respectively, P = 0.002); alkaline phosphatase The levels of collagen type Ⅰ and osteocalcin were significantly lower in patients with congenital scoliosis (44.69 IU / mg and 51.38 IU / mg, P = 0.019; 226.34 ng / mg and 249.93 ng / mg, P = 0.014 and 7.41 ng / mg and 8.87 ng / mg, P = 0.049). Osteoblasts from patients with NF1 scoliosis showed relatively active proliferative activity (multiples of 3.34 and 2.70, respectively, P = 0.049). Conclusions The expression of neuroblastoma protein in patients with NF1 scoliosis is decreased, while its function is obviously deficient. Osteoblasts dysfunction may be the common basis for various skeletal abnormalities, such as altered bone malnutrition and decreased bone mineral density.