文章简介尼曼—匹克C型疾病是由NPC1或者NPC2基因突变造成的一类脂类代谢异常疾病。NPC1和NPC2蛋白在胆固醇的细胞内转运过程中发挥了重要功能,同时NPC1还作为埃博拉病毒位于细胞内的受体参与了病毒的入侵过程。本研究首次报道了人源胆固醇转运蛋白NPC1的4.4埃分辨率冷冻电镜结构,并通过大量生化分析探讨 Article Outline Niemann-Pick C-type disease is a type of lipid metabolism disorder caused by NPC1 or NPC2 gene mutation. NPC1 and NPC2 proteins play an important role in the intracellular transport of cholesterol, and NPC1 is also involved in the virus invasion process as a receptor located in the cell of Ebola virus. This study reports for the first time the human cholesterol transporter NPC1 resolution of 4.4 angstrom electron microscopy structure, and through a large number of biochemical analysis