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首页> 外文期刊>The FEBS journal >Leukocyte adhesion deficiency II
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Leukocyte adhesion deficiency II

机译:白细胞黏附缺乏症II

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摘要

Leukocyte adhesion deficiency II (LAD II) belongs to a group of human congenital diseases in which the interactions of leukocytes with the vascular endothelium are strongly impaired. LAD II is based on a defect in the synthesis of fucosylated glycostructures. This leads to an immunodeficiency owing to the absence of functional selectin ligands and to strong psychomotor defects, as a result of as-yet unknown reasons. In this review we focused on the current controversies, and open questions that have arisen from recent studies on the genetic defect, therapy and the basis of psychomotor defects in LAD II.
机译:白细胞粘附缺乏症II(LAD II)属于人类先天性疾病,其中白细胞与血管内皮的相互作用受到严重损害。 LAD II基于岩藻糖基化糖结构合成的缺陷。由于尚不知道的原因,由于缺乏功能选择蛋白配体和强烈的精神运动缺陷而导致免疫缺陷。在这篇综述中,我们集中于当前的争议,以及有关LAD II的遗传缺陷,治疗和精神运动缺陷基础的最新研究引起的开放性问题。

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