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The Niemann-Pick C lesion and its relationship to the intracellular distribution and utilization of LDL cholesterol.

作者信息

Pentchev P G, Brady R O, Blanchette-Mackie E J, Vanier M T, Carstea E D, Parker C C, Goldin E, Roff C F

机构信息

Molecular and Cellular Pathophysiology Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.

出版信息

Biochim Biophys Acta. 1994 Feb 22;1225(3):235-43. doi: 10.1016/0925-4439(94)90001-9.

DOI:10.1016/0925-4439(94)90001-9
PMID:8312368
Abstract
摘要

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The Niemann-Pick C lesion and its relationship to the intracellular distribution and utilization of LDL cholesterol.尼曼-匹克C型病变及其与低密度脂蛋白胆固醇细胞内分布和利用的关系。
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Changes in cholesterol metabolism in cultured fibroblasts from patients with Niemann-Pick disease.尼曼-匹克病患者培养成纤维细胞中胆固醇代谢的变化。
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Regulation of intracellular cholesterol metabolism is defective in lymphoblasts from Niemann-Pick type C and type D patients.尼曼-匹克C型和D型患者的淋巴母细胞中，细胞内胆固醇代谢的调节存在缺陷。
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The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts.在尼曼-皮克C型成纤维细胞中，低密度脂蛋白衍生胆固醇的细胞内转运存在缺陷。
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Group C Niemann-Pick disease: faulty regulation of low-density lipoprotein uptake and cholesterol storage in cultured fibroblasts.C型尼曼-匹克病：培养的成纤维细胞中低密度脂蛋白摄取和胆固醇储存的调节异常。
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[Impaired cholesterol esterification in Niemann-Pick disease model mouse].
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Type C Niemann-Pick disease: dimethyl sulfoxide moderates abnormal LDL-cholesterol processing in mutant fibroblasts.
Biochim Biophys Acta. 1989 Nov 28;1006(2):219-26. doi: 10.1016/0005-2760(89)90200-2.

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