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纯合子II型高脂蛋白血症患者皮肤成纤维细胞中固醇合成及羟甲基戊二酰辅酶A还原酶的调控

Control of sterol synthesis and of hydroxymethylglutaryl CoA reductase in skin fibroblasts grown from patients with homozygous type II hyperlipoproteinemia.

作者信息

Avigan J, Bhathena S J, Schreiner M E

出版信息

J Lipid Res. 1975 Mar;16(2):151-4.

PMID:236350
Abstract

In skin fibroblasts grown from four children with a homozygous form of type II hyperlipoproteinemia, the feedback control of sterol synthesis and the inhibitory effect on hydroxymethylglutaryl (HMG) CoA reductase activity by serum or low density lipoprotein were present, though diminished compared with the effects in normal fibroblasts. Stimulation of HMG CoA reductase by insulin and inhibition of acetyl CoA carboxylase by serum lipids were not impaired in these type II cells, indicating a degree of specificity in the abnormal response of the reductase. A rapid and convenient method for isolation of mevalonolactone in the course of the assay of HMG CoA reductase is described.

摘要

在从四名患有纯合子型II型高脂蛋白血症的儿童身上培养的皮肤成纤维细胞中，存在固醇合成的反馈控制以及血清或低密度脂蛋白对羟甲基戊二酰（HMG）辅酶A还原酶活性的抑制作用，尽管与正常成纤维细胞中的作用相比有所减弱。胰岛素对HMG辅酶A还原酶的刺激以及血清脂质对乙酰辅酶A羧化酶的抑制在这些II型细胞中并未受损，这表明还原酶的异常反应具有一定程度的特异性。本文描述了一种在HMG辅酶A还原酶测定过程中分离甲羟戊酸内酯的快速简便方法。

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1
Control of sterol synthesis and of hydroxymethylglutaryl CoA reductase in skin fibroblasts grown from patients with homozygous type II hyperlipoproteinemia.纯合子II型高脂蛋白血症患者皮肤成纤维细胞中固醇合成及羟甲基戊二酰辅酶A还原酶的调控
J Lipid Res. 1975 Mar;16(2):151-4.
2
Regulation of lipid synthesis by low density lipoproteins in cultured skin fibroblasts in homozygous familial hypercholesterolemia.纯合子家族性高胆固醇血症患者培养的皮肤成纤维细胞中低密度脂蛋白对脂质合成的调节作用
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Arch Biochem Biophys. 1986 Jan;244(1):310-22. doi: 10.1016/0003-9861(86)90120-7.
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Biochim Biophys Acta. 1978 Mar 30;528(3):445-55. doi: 10.1016/0005-2760(78)90034-6.
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Regulation of low density lipoprotein receptor and 3-hydroxy-3-methyl-glutaryl-CoA reductase activities are differentially affected in Niemann-Pick type C and type D fibroblasts.在尼曼-匹克C型和D型成纤维细胞中，低密度脂蛋白受体和3-羟基-3-甲基戊二酰辅酶A还原酶活性的调节受到不同影响。
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Inhibition, post-hydroxymethylglutaryl-CoA regulation and relation to cell growth of cholesterol biosynthesis in cultured human skin fibroblasts.培养的人皮肤成纤维细胞中胆固醇生物合成的抑制、羟甲基戊二酰辅酶A调节及其与细胞生长的关系
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引用本文的文献

1
Receptor-mediated low-density lipoprotein catabolism.受体介导的低密度脂蛋白分解代谢
Klin Wochenschr. 1982 Apr 1;60(7):319-25. doi: 10.1007/BF01721621.
2
The effects of amphotericin B on lipid metabolism in cultured human skin fibroblasts.两性霉素B对培养的人皮肤成纤维细胞脂质代谢的影响。
In Vitro Cell Dev Biol. 1985 Jan;21(1):26-31. doi: 10.1007/BF02620910.
3
Preparation of delipidized serum protein for use in cell culture systems.用于细胞培养系统的脱脂血清蛋白的制备。
In Vitro. 1976 Aug;12(8):554-7. doi: 10.1007/BF02797438.
4
Diagnosing familial hypercholesterolaemia in childhood.儿童家族性高胆固醇血症的诊断
Br Med J. 1977 Oct 8;2(6092):957-8. doi: 10.1136/bmj.2.6092.957-a.
5
Perinatal development of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in rat lung, liver and brain.大鼠肺、肝和脑中3-羟基-3-甲基戊二酰辅酶A还原酶活性的围产期发育。
Lipids. 1979 May;14(5):447-50. doi: 10.1007/BF02533460.
6
Essential role for mevalonate synthesis in DNA replication.甲羟戊酸合成在DNA复制中的关键作用。
Proc Natl Acad Sci U S A. 1979 Oct;76(10):5056-60. doi: 10.1073/pnas.76.10.5056.
7
3-Hydroxy-3-methylglutaryl coenzyme A reductase: regulation of enzymatic activity by phosphorylation and dephosphorylation.3-羟基-3-甲基戊二酰辅酶A还原酶：通过磷酸化和去磷酸化对酶活性的调节
Proc Natl Acad Sci U S A. 1978 Aug;75(8):3678-82. doi: 10.1073/pnas.75.8.3678.
8
Regulation of cholesterol biosynthesis in HeLa S3G cells by serum lipoproteins: dexamethasone-mediated interference with suppression of 3-hydroxy-3-methylglutaryl coenzyme A reductase.血清脂蛋白对HeLa S3G细胞胆固醇生物合成的调节：地塞米松介导的对3-羟基-3-甲基戊二酰辅酶A还原酶抑制作用的干扰。
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9
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