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揭开谜团……黏附分子缺陷

Demystified...adhesion molecule deficiencies.

作者信息

Inwald D, Davies E G, Klein N

机构信息

Portex Department of Anaesthesia, Intensive Care and Respiratory Medicine, Institute of Child Health, London, UK.

出版信息

Mol Pathol. 2001 Feb;54(1):1-7. doi: 10.1136/mp.54.1.1.

DOI:10.1136/mp.54.1.1
PMID:11212883
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1186993/
Abstract

The basic physiology of leucocyte emigration from the intravascular space into the tissues is now known to be dependent on a class of cell surface molecules that have come to be known as adhesion molecules. Many cell-cell interactions are dependent on adhesion and signal transduction via the various adhesion molecules, particularly the integrins. The study of the functions of these molecules has been enhanced by the development of blocking and activating monoclonal antibodies, knockout mice, and by the rare "experiments of nature" in the human population, in whom there is absence or dysfunction of one of the adhesion molecules. This review describes these leucocyte adhesion defects and discusses how they have provided important insights into the function of these molecules.

摘要

目前已知,白细胞从血管内空间迁移至组织的基本生理过程依赖于一类现已被称为黏附分子的细胞表面分子。许多细胞间相互作用依赖于通过各种黏附分子,尤其是整合素进行的黏附及信号转导。阻断性和活化性单克隆抗体、基因敲除小鼠的研制,以及人类中罕见的“自然实验”(即存在某一种黏附分子缺失或功能障碍的个体),促进了对这些分子功能的研究。本综述描述了这些白细胞黏附缺陷,并讨论了它们如何为这些分子的功能提供了重要见解。

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本文引用的文献

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A novel point mutation in CD18 causing the expression of dysfunctional CD11/CD18 leucocyte integrins in a patient with leucocyte adhesion deficiency (LAD).CD18基因中的一种新型点突变导致白细胞黏附缺陷症(LAD)患者表达功能失调的CD11/CD18白细胞整合素。
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A novel CD18 genomic deletion in a patient with severe leucocyte adhesion deficiency: a possible CD2/lymphocyte function-associated antigen-1 functional association in humans.一名严重白细胞黏附缺陷患者中一种新的CD18基因缺失:人类中可能存在的CD2/淋巴细胞功能相关抗原-1功能关联
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Platelet GPIIb-IIIa blockers.血小板糖蛋白IIb-IIIa阻滞剂
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A novel leukocyte adhesion deficiency caused by expressed but nonfunctional beta2 integrins Mac-1 and LFA-1.由表达但无功能的β2整合素Mac-1和LFA-1引起的一种新型白细胞黏附缺陷症。
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Truncation of glycoprotein (GP) IIIa (616-762) prevents complex formation with GPIIb: novel mutation in exon 11 of GPIIIa associated with thrombasthenia.糖蛋白(GP)IIIa(616 - 762)的截短会阻止其与GPIIb形成复合物:与血小板无力症相关的GPIIIa外显子11中的新突变。
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