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四种胆固醇感知蛋白。

Four cholesterol-sensing proteins.

作者信息

Lange Y, Steck T L

机构信息

Department of Pathology, Rush Presbyterian St Luke's Medical Center, Chicago, IL 60612, USA.

出版信息

Curr Opin Struct Biol. 1998 Aug;8(4):435-9. doi: 10.1016/s0959-440x(98)80119-x.

DOI:10.1016/s0959-440x(98)80119-x
PMID:9729733
Abstract

What is the connection among the following three medical conditions: Niemann-Pick type C disease (a cause of mental retardation and early death), systemic lipidosis (in which an obscure side effect of numerous drugs transforms lysosomes into lamellar bodies), and holoprosencephaly (a catastrophe in embryonic development)? Recent evidence suggests that the pathogenesis in each use involves impaired sensing of cellular cholesterol.

摘要

以下三种医学病症之间有什么联系

尼曼-皮克C型病(智力发育迟缓及过早死亡的一个病因)、系统性脂质沉积症(众多药物的一种不明副作用会将溶酶体转变为板层小体)和前脑无裂畸形(胚胎发育中的一种严重病变)?最近的证据表明,每种病症的发病机制都涉及细胞胆固醇感知受损。

相似文献

1
Four cholesterol-sensing proteins.四种胆固醇感知蛋白。
Curr Opin Struct Biol. 1998 Aug;8(4):435-9. doi: 10.1016/s0959-440x(98)80119-x.
2
Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis.尼曼-匹克C1病基因:与胆固醇稳态调节因子的同源性
Science. 1997 Jul 11;277(5323):228-31. doi: 10.1126/science.277.5323.228.
3
Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene.尼曼-匹克C病的小鼠模型:胆固醇稳态基因的突变
Science. 1997 Jul 11;277(5323):232-5. doi: 10.1126/science.277.5323.232.
4
Niemann-Pick type-C disease: deficient intracellular transport of exogenously derived cholesterol.尼曼-匹克C型病:外源性胆固醇的细胞内转运缺陷。
Am J Med Genet. 1992 Feb 15;42(4):593-8. doi: 10.1002/ajmg.1320420433.
5
Cholesterol: stuck in traffic.胆固醇:陷入困境。
Nat Cell Biol. 1999 Jun;1(2):E37-8. doi: 10.1038/10030.
6
Related membrane domains in proteins of sterol sensing and cell signaling provide a glimpse of treasures still buried within the dynamic realm of intracellular metabolic regulation.
Curr Opin Lipidol. 1998 Apr;9(2):137-40. doi: 10.1097/00041433-199804000-00010.
7
Topological analysis of Niemann-Pick C1 protein reveals that the membrane orientation of the putative sterol-sensing domain is identical to those of 3-hydroxy-3-methylglutaryl-CoA reductase and sterol regulatory element binding protein cleavage-activating protein.尼曼-皮克病C1蛋白的拓扑分析表明,假定的固醇感应结构域的膜取向与3-羟基-3-甲基戊二酰辅酶A还原酶和固醇调节元件结合蛋白裂解激活蛋白的膜取向相同。
J Biol Chem. 2000 Aug 11;275(32):24367-74. doi: 10.1074/jbc.M002184200.
8
Niemann-Pick type C disease and intracellular cholesterol trafficking.尼曼-匹克C型病与细胞内胆固醇转运
J Biol Chem. 2005 Jun 3;280(22):20917-20. doi: 10.1074/jbc.R400040200. Epub 2005 Apr 14.
9
The structure and function of the Niemann-Pick C1 protein.尼曼-皮克C1蛋白的结构与功能。
Mol Genet Metab. 2000 Sep-Oct;71(1-2):175-81. doi: 10.1006/mgme.2000.3061.
10
The role of cholesterol in Shh signaling and teratogen-induced holoprosencephaly.胆固醇在 Sonic Hedgehog(Shh)信号传导及致畸剂诱导的前脑无裂畸形中的作用
Cell Mol Life Sci. 2000 Nov;57(12):1709-19. doi: 10.1007/PL00000653.

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Cholesterol metabolism pathways - are the intermediates more important than the products?胆固醇代谢途径——中间产物比产物更重要吗?
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Genetics. 2001 Mar;157(3):1117-40. doi: 10.1093/genetics/157.3.1117.
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A C. elegans patched gene, ptc-1, functions in germ-line cytokinesis.一种秀丽隐杆线虫的patched基因,ptc-1,在生殖系胞质分裂中发挥作用。
Genes Dev. 2000 Aug 1;14(15):1933-44.
6
Niemann-Pick C1 disease: the I1061T substitution is a frequent mutant allele in patients of Western European descent and correlates with a classic juvenile phenotype.尼曼-皮克C1病:I1061T替代是西欧血统患者中常见的突变等位基因,且与典型的青少年表型相关。
Am J Hum Genet. 1999 Nov;65(5):1321-9. doi: 10.1086/302626.
7
The role of the 3-hydroxy 3-methylglutaryl coenzyme A reductase cytosolic domain in karmellae biogenesis.3-羟基-3-甲基戊二酰辅酶A还原酶胞质结构域在卡麦拉体生物发生中的作用。
Mol Biol Cell. 1999 Oct;10(10):3409-23. doi: 10.1091/mbc.10.10.3409.