| NPC蛋白研究进展 |
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| 引用本文： | 赵玄女,徐亮,冯明业,姚雪彪.NPC蛋白研究进展[J].济宁师范专科学校学报,2004,25(6):6-8,11. |
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| 作者姓名： | 赵玄女 徐亮 冯明业 姚雪彪 |
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| 作者单位： | 中国科学技术大学细胞动力学实验室 合肥230027
(赵玄女，徐亮，冯明业)，中国科学技术大学细胞动力学实验室 合肥230027(姚雪彪) |
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| 摘 要： | C型尼曼氏病(Niemann-Picktype C，NPC)是一种致命的神经退行性失调疾病，常见于儿童早期发作。其特征是患者细胞中胆固醇和鞘糖脂等脂类沉积在晚期胞内体／溶酶体中。NPC症状是由NPCI或NPC2的功能缺陷引起的。NPCI是一个跨膜蛋白，目前认为它参与内吞后的底物向高尔基复合体或胞内其它位点运输，在脂类物质循环和平衡控制机制中起重要作用。NPC2是一个可溶性溶酶体蛋白，可结合胆固醇。目前尚不清楚NPCI和NPC2如何调控溶酶体等细胞器的确切功能。
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| 关 键 词： | NPC1 NPCC2 晚期胞内体 溶酶体 胆固醇 脂类运输 |
| 文章编号： | 1004-1877(2004)06-0006-03 |
Progress on Niemann- Pick C Proteins |
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| Zhao Xuannv,Xu Liang,Feng Mingye,Yao Xuebiao.Progress on Niemann- Pick C Proteins[J].Journal of Jining Teachers College,2004,25(6):6-8,11. |
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| Authors: | Zhao Xuannv Xu Liang Feng Mingye Yao Xuebiao |
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| Abstract: | Niemann-Pick type C, or NPC for short, is an early childhood disease exhibiting progressive neurological degeneration. At the cellular level, the disease is a result of improper trafficking of lipids such as cholesterol and glycosphingolipids (GSLs) to a late endosome/ lysosome compartment. NPC cells have defects in the proteins NPC1 or NPC2. NPC1 is a transmembrane protein believed involved in retroendocytic shuttling of substrate(s) to the Golgi and possibly elsewhere in cells as part of an essential recycling/ homeostatic control mechanism. NPC2 is a soluble lysosomal protein known to bind cholesterol. The precise roles of NPC1 and NPC2 in late endosomal/ lysosomal function remain unclear. |
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| Keywords: | NPC1 NPC2 late endosome lysosome cholesterol lipid trafficking |
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