| 不同病程的肝豆状核变性患者培养细胞中的溶酶体变化 |
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| 引用本文： | 李采娟,刘道宽,陈秀珍,左伋,夏蓓莉,李乃忠,毛明珍.不同病程的肝豆状核变性患者培养细胞中的溶酶体变化[J].复旦学报(医学版),1988(2). |
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| 作者姓名： | 李采娟 刘道宽 陈秀珍 左伋 夏蓓莉 李乃忠 毛明珍 |
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| 作者单位： | 上海医科大学基础医学部生物学教研室
(李采娟，陈秀珍，左伋，夏蓓莉)，上海医科大学神经病学研究所
(刘道宽，李乃忠)，上海医科大学基础医学部电镜室(毛明珍) |
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| 基金项目： | 中国科学院科学基金(科基生85～481) |
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| 摘 要： | 本文以培养的皮肤成纤维细胞为离体细胞模型,用电镜观察8例肝豆状核变性(HLD)患者,10例正常对照者的培养细胞。经图像分析仪定量测定的结果表明,早期患者细胞溶酶体的体积密度、数密度与对照者无显著差异,但溶酶体的体积密度与病情的进展呈正相关关系(r=0.98,tr=12.06,p<0.01)。由此讨论了溶酶体在病理发生中的作用。
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| 关 键 词： | 肝豆状核变性 纤维母细胞 溶酶体 图像立体分析 |
LYSOSOMAL CHANGES OF CULTURED FIBROBLASTS WITH THE PROFRESSION OF HEPATOLENTICQLAR DEGENEYATION |
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| Li Caijuan,Liu Daokuan,Chen Xiuzhen,Zuo Ji,Xia Beili,Li Naizhong,Mao Mingzheng.LYSOSOMAL CHANGES OF CULTURED FIBROBLASTS WITH THE PROFRESSION OF HEPATOLENTICQLAR DEGENEYATION[J].Fudan University Journal of Medical Sciences,1988(2). |
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| Authors: | Li Caijuan Liu Daokuan Chen Xiuzhen Zuo Ji Xia Beili Li Naizhong Mao Mingzheng |
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| Abstract: | The main defect in copper metabolism might appeared in lysosomes of the hepatocytes. Lysosomal changes of cultured skin fibroblasts were studied by electron microscopy and stereologic analyses. Results reveal that there are no significant differences in lysosomic volumetric density (Vy) and numerical density (Nv) between early HLD patients and controls. However, the Vv of lysosomes increased with progression of the disease (r=0.98, tr = 12.06, p<0.01). These data suggest a possible role of lysosomal changes in the pathogenesis of HLD. |
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| Keywords: | hepatolenticular degeneration fibroblasts lysosomes stereological analysis |
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