来自一名正常受试者和一名纯合子家族性高胆固醇血症患者的长期淋巴细胞系中低密度脂蛋白的结合、内化及水解作用
Binding, internalization, and hydrolysis of low density lipoprotein in long-term lymphoid cell lines from a normal subject and a patient with homozygous familial hypercholesterolemia.
作者信息
Ho Y K, Brown M S, Kayden H J, Goldstein J L
出版信息
J Exp Med. 1976 Aug 1;144(2):444-55. doi: 10.1084/jem.144.2.444.
Abstract
Long-term established human lymphoid cells were shown to possess high affinity cell surface receptors for low density lipoprotein (LDL), the major cholesterol-carrying protein in human plasma. Binding of LDL to these receptors was followed by internalization of the lipoprotein and hydrolysis of its protein and cholesteryl ester components. Cultured lymphocytes from a patient with the homozygous form of familial hypercholesterolemia lacked cell surface LDL receptors and therefore failed to take up and degrade the lipoprotein with high affinity. Cultured human lymphocytes should prove useful for further studies of: (a) the relation between cholesterol metabolism and cellular function and (b) the mechanism by which LDL binding at the cell surface leads to internalization of the lipoprotein.
摘要
长期建立的人类淋巴细胞被证明具有对低密度脂蛋白（LDL）的高亲和力细胞表面受体，LDL是人类血浆中主要的携带胆固醇的蛋白质。LDL与这些受体结合后，脂蛋白会被内化，其蛋白质和胆固醇酯成分会被水解。来自纯合子家族性高胆固醇血症患者的培养淋巴细胞缺乏细胞表面LDL受体，因此无法以高亲和力摄取和降解脂蛋白。培养的人类淋巴细胞应被证明对以下进一步研究有用：（a）胆固醇代谢与细胞功能之间的关系；（b）细胞表面LDL结合导致脂蛋白内化的机制。
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Binding, internalization, and hydrolysis of low density lipoprotein in long-term lymphoid cell lines from a normal subject and a patient with homozygous familial hypercholesterolemia.来自一名正常受试者和一名纯合子家族性高胆固醇血症患者的长期淋巴细胞系中低密度脂蛋白的结合、内化及水解作用
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本文引用的文献
1
Protein measurement with the Folin phenol reagent.使用福林酚试剂进行蛋白质测定。
J Biol Chem. 1951 Nov;193(1):265-75.
2
The interaction of IgE with rat basophilic leukemia cells. I. Evidence for specific binding of IgE.免疫球蛋白E（IgE）与大鼠嗜碱性白血病细胞的相互作用。I. IgE特异性结合的证据。
J Exp Med. 1974 Mar 1;139(3):600-16. doi: 10.1084/jem.139.3.600.
3
4
Esterification of low density lipoprotein cholesterol in human fibroblasts and its absence in homozygous familial hypercholesterolemia.人成纤维细胞中低密度脂蛋白胆固醇的酯化及其在纯合子家族性高胆固醇血症中的缺失。
Proc Natl Acad Sci U S A. 1974 Nov;71(11):4288-92. doi: 10.1073/pnas.71.11.4288.
5
6
Lipoprotein uptake and metabolism by rat aortic smooth muscle cells in tissue culture.组织培养中大鼠主动脉平滑肌细胞对脂蛋白的摄取与代谢
Circ Res. 1974 Jul;35(1):136-50. doi: 10.1161/01.res.35.1.136.
7
Familial hypercholesterolemia: defective binding of lipoproteins to cultured fibroblasts associated with impaired regulation of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity.家族性高胆固醇血症：脂蛋白与培养的成纤维细胞结合缺陷，与3-羟基-3-甲基戊二酰辅酶A还原酶活性调节受损有关。
Proc Natl Acad Sci U S A. 1974 Mar;71(3):788-92. doi: 10.1073/pnas.71.3.788.
8
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J Biol Chem. 1974 Feb 10;249(3):789-96.
9
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Biochemistry. 1976 Feb 10;15(3):521-8. doi: 10.1021/bi00648a011.
10
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