相似文献
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Niemann-Pick type C disease: molecular mechanisms and potential therapeutic approaches.尼曼-匹克 C 型病：分子机制与潜在治疗方法。
J Neurochem. 2011 Mar;116(5):789-95. doi: 10.1111/j.1471-4159.2010.06976.x. Epub 2011 Jan 7.
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Niemann-Pick C1 functions independently of Niemann-Pick C2 in the initial stage of retrograde transport of membrane-impermeable lysosomal cargo.尼曼-匹克 C1 蛋白在膜不渗透溶酶体货物逆行运输的初始阶段独立于尼曼-匹克 C2 蛋白发挥作用。
J Biol Chem. 2010 Feb 12;285(7):4983-94. doi: 10.1074/jbc.M109.037622. Epub 2009 Dec 10.
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Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin.尼曼-皮克C型病与环糊精对溶酶体胆固醇的动员作用
J Lipid Res. 2014 Aug;55(8):1609-21. doi: 10.1194/jlr.R047837. Epub 2014 Mar 24.
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Molecular dynamics study with mutation shows that N-terminal domain structural re-orientation in Niemann-Pick type C1 is required for proper alignment of cholesterol transport.分子动力学突变研究表明，尼曼-匹克 C1 型中 N 端结构重排对于胆固醇转运的正确排列是必需的。
J Neurochem. 2021 Mar;156(6):967-978. doi: 10.1111/jnc.15150. Epub 2020 Sep 16.
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The unique case of the Niemann-Pick type C cholesterol storage disorder.尼曼-匹克C型胆固醇贮积症的独特病例。
Pediatr Endocrinol Rev. 2014 Sep;12 Suppl 1:166-75.
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Reduction of TMEM97 increases NPC1 protein levels and restores cholesterol trafficking in Niemann-pick type C1 disease cells.降低TMEM97可提高NPC1蛋白水平并恢复尼曼-匹克C1型病细胞中的胆固醇转运。
Hum Mol Genet. 2016 Aug 15;25(16):3588-3599. doi: 10.1093/hmg/ddw204. Epub 2016 Jul 4.
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Abnormal accumulation and recycling of glycoproteins visualized in Niemann-Pick type C cells using the chemical reporter strategy.利用化学报告策略观察到尼曼-匹克 C 型细胞中糖蛋白的异常积累和再循环。
Proc Natl Acad Sci U S A. 2013 Jun 18;110(25):10207-12. doi: 10.1073/pnas.1221105110. Epub 2013 Jun 3.
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ABCA1-dependent mobilization of lysosomal cholesterol requires functional Niemann-Pick C2 but not Niemann-Pick C1 protein.ATP结合盒转运蛋白A1（ABCA1）依赖的溶酶体胆固醇转运需要功能性的尼曼-皮克C2蛋白，但不需要尼曼-皮克C1蛋白。
Biochim Biophys Acta. 2012 Mar;1821(3):396-404. doi: 10.1016/j.bbalip.2011.11.013. Epub 2011 Dec 10.
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Altered vitamin E status in Niemann-Pick type C disease.尼曼-匹克 C 型病患者维生素 E 状态的改变。
J Lipid Res. 2011 Jul;52(7):1400-10. doi: 10.1194/jlr.M015560. Epub 2011 May 5.
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Molecular analysis of NPC1 and NPC2 gene in 34 Niemann-Pick C Italian patients: identification and structural modeling of novel mutations.34例意大利尼曼-匹克C型患者NPC1和NPC2基因的分子分析：新突变的鉴定与结构建模
Neurogenetics. 2009 Jul;10(3):229-39. doi: 10.1007/s10048-009-0175-3. Epub 2009 Feb 28.
引用本文的文献
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Dynamic mode decomposition for analysis and prediction of metabolic oscillations from time-lapse imaging of cellular autofluorescence.用于从细胞自发荧光延时成像分析和预测代谢振荡的动态模式分解
Sci Rep. 2025 Jul 2;15(1):23489. doi: 10.1038/s41598-025-07255-4.
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Itraconazole and posaconazole, inhibitors of NPC1 sterol transport, act as pharmacological chaperones after washout.伊曲康唑和泊沙康唑作为NPC1固醇转运抑制剂，在洗脱后可作为药理伴侣发挥作用。
J Biol Chem. 2025 Jul;301(7):110370. doi: 10.1016/j.jbc.2025.110370. Epub 2025 Jun 16.
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Advances and therapeutic opportunities in visual cycle modulation.视觉循环调节的进展与治疗机遇
Prog Retin Eye Res. 2025 May;106:101360. doi: 10.1016/j.preteyeres.2025.101360. Epub 2025 Apr 23.
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Bafilomycin A1 Inhibits HIV-1 Infection by Disrupting Lysosomal Cholesterol Transport.巴氟霉素 A1 通过破坏溶酶体胆固醇转运抑制 HIV-1 感染。
Viruses. 2024 Aug 29;16(9):1374. doi: 10.3390/v16091374.
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Novel opportunities from bioimaging to understand the trafficking and maturation of intracellular pulmonary surfactant and its role in lung diseases.从生物成像中探索新机遇，以了解细胞内肺表面活性剂的运输和成熟过程及其在肺部疾病中的作用。
Front Immunol. 2023 Aug 10;14:1250350. doi: 10.3389/fimmu.2023.1250350. eCollection 2023.
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Different solubilizing ability of cyclodextrin derivatives for cholesterol in Niemann-Pick disease type C treatment.不同环糊精衍生物对尼曼-匹克病 C 型治疗中胆固醇的增溶能力。
Clin Transl Med. 2023 Aug;13(8):e1350. doi: 10.1002/ctm2.1350.
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Alterations in Cholesterol and Phosphoinositides Levels in the Intracellular Cholesterol Trafficking Disorder NPC.细胞内胆固醇运输障碍 NPC 中胆固醇和磷酯酰肌醇水平的改变。
Adv Exp Med Biol. 2023;1422:143-165. doi: 10.1007/978-3-031-21547-6_5.
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Proteomic analysis of s-acylated proteins in human retinal pigment epithelial cells and the role of palmitoylation of Niemann-Pick type C1 protein in cholesterol transport.人视网膜色素上皮细胞中S-酰化蛋白的蛋白质组学分析以及尼曼-匹克C1型蛋白的棕榈酰化在胆固醇转运中的作用。
Front Aging Neurosci. 2022 Oct 3;14:965943. doi: 10.3389/fnagi.2022.965943. eCollection 2022.
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Niemann-Pick Type C Proteins Are Required for Sterol Transport and Appressorium-Mediated Plant Penetration of .尼曼-匹克 C 型蛋白对于固醇运输和附着胞介导的. 植物穿透是必需的。
mBio. 2022 Oct 26;13(5):e0223622. doi: 10.1128/mbio.02236-22. Epub 2022 Sep 26.
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The Cholesterol Transport Inhibitor U18666A Interferes with Pseudorabies Virus Infection.胆固醇转运抑制剂 U18666A 干扰伪狂犬病毒感染。
Viruses. 2022 Jul 14;14(7):1539. doi: 10.3390/v14071539.
本文引用的文献
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Identification of surface residues on Niemann-Pick C2 essential for hydrophobic handoff of cholesterol to NPC1 in lysosomes.鉴定溶酶体中尼曼-匹克 C2 向 NPC1 转移胆固醇所必需的表面残基。
Cell Metab. 2010 Aug 4;12(2):166-73. doi: 10.1016/j.cmet.2010.05.016.
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Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the Niemann-Pick type C1 mouse and markedly prolongs life.每周给予环糊精治疗可使尼曼-匹克 C1 型小鼠几乎所有器官的胆固醇代谢恢复正常，并显著延长其寿命。
Pediatr Res. 2010 Oct;68(4):309-15. doi: 10.1203/PDR.0b013e3181ee4dd2.
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Thiadiazole carbamates: potent inhibitors of lysosomal acid lipase and potential Niemann-Pick type C disease therapeutics.噻二唑基氨基甲酸酯：溶酶体酸性脂肪酶的有效抑制剂和潜在的尼曼-匹克 C 型疾病治疗药物。
J Med Chem. 2010 Jul 22;53(14):5281-9. doi: 10.1021/jm100499s.
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Increased expression of the lysosomal cholesterol transporter NPC1 in Alzheimer's disease.溶酶体胆固醇转运蛋白NPC1在阿尔茨海默病中的表达增加。
Biochim Biophys Acta. 2010 Aug;1801(8):831-8. doi: 10.1016/j.bbalip.2010.05.005. Epub 2010 May 16.
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Niemann-Pick type C cells show cholesterol dependent decrease of APP expression at the cell surface and its increased processing through the beta-secretase pathway.尼曼-皮克C型细胞显示出细胞表面APP表达依赖胆固醇的减少以及通过β-分泌酶途径对其加工的增加。
Biochim Biophys Acta. 2010 Jul-Aug;1802(7-8):682-91. doi: 10.1016/j.bbadis.2010.05.006. Epub 2010 May 20.
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Improvement in lipid and protein trafficking in Niemann-Pick C1 cells by correction of a secondary enzyme defect.通过纠正次要酶缺陷改善尼曼-匹克 C1 细胞中的脂质和蛋白质转运。
Traffic. 2010 May;11(5):601-15. doi: 10.1111/j.1600-0854.2010.01046.x. Epub 2010 Feb 22.
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Endocytosis of beta-cyclodextrins is responsible for cholesterol reduction in Niemann-Pick type C mutant cells.β-环糊精的内吞作用是导致尼曼-匹克 C 型突变细胞胆固醇降低的原因。
Proc Natl Acad Sci U S A. 2010 Mar 23;107(12):5477-82. doi: 10.1073/pnas.0914309107. Epub 2010 Mar 8.
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Cholesterol accumulation in Niemann Pick type C (NPC) model cells causes a shift in APP localization to lipid rafts.胆固醇在尼曼-匹克 C 型（NPC）模型细胞中的积累导致 APP 定位向脂筏转移。
Biochem Biophys Res Commun. 2010 Mar 12;393(3):404-9. doi: 10.1016/j.bbrc.2010.02.007. Epub 2010 Feb 6.
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Lipids on trial: the search for the offending metabolite in Niemann-Pick type C disease.脂质受审：在尼曼-匹克 C 型疾病中寻找罪魁祸首代谢物。
Traffic. 2010 Apr;11(4):419-28. doi: 10.1111/j.1600-0854.2010.01032.x. Epub 2010 Jan 6.
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Miglustat in adult and juvenile patients with Niemann-Pick disease type C: long-term data from a clinical trial.米格列醇治疗成年和青少年尼曼-匹克病 C 型患者：临床试验的长期数据。
Mol Genet Metab. 2010 Apr;99(4):351-7. doi: 10.1016/j.ymgme.2009.12.006. Epub 2009 Dec 31.
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