Department of Pediatrics, Institute of Molecular Medicine Angelo Nocivelli, University of Brescia, Brescia, Italy.
Eur J Immunol. 2013 Jun;43(6):1436-40. doi: 10.1002/eji.201243155.
This Viewpoint gives an overview of the inherited disorders that are characterized by defects of leukocyte trafficking. Three paradigmatic diseases have been selected: warts, hypogammaglobulinemia, infections, and myelokathexis; Wiskott-Aldrich; and leukocyte adhesion deficiency syndromes. These conditions encompass defects in the steps that are required for leukocyte motility: from the response to chemokines, which is altered in warts, hypogammaglobulinemia, infections, and myelokathexis syndrome, to the impairment of leukocyte adhesion and migration found in leukocyte adhesion deficiency syndrome, and finally to abnormal actin filament formation, which is affected in leukocytes of Wiskott-Aldrich syndrome patients.
本文观点概述了以白细胞运输缺陷为特征的遗传性疾病。选择了三种典型疾病：疣、低丙种球蛋白血症、感染和骨髓发育不良；Wiskott-Aldrich 综合征；以及白细胞黏附缺陷综合征。这些病症包括白细胞运动所需步骤的缺陷：从对趋化因子的反应（疣、低丙种球蛋白血症、感染和骨髓发育不良综合征中发生改变），到白细胞黏附缺陷综合征中发现的白细胞黏附和迁移受损，最后是异常的肌动蛋白丝形成，Wiskott-Aldrich 综合征患者的白细胞受其影响。
