Arnaout M Amin
Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Immunol Rev. 2025 Jan;329(1):e13433. doi: 10.1111/imr.13433. Epub 2024 Dec 26.
Innate immune cells perform vital tasks in detecting, seeking, and eliminating invading pathogens, thus ensuring host survival. However, loss of function of these cells or their overactive response to tissue injury often causes serious ailments. It is, therefore, crucial to understand at a basic level how these cells function in health and disease. A major step toward this goal came from studies I conducted in the late 1970s investigating the cause of life-threatening bacterial infections in a pediatric patient. This work led us to trace this disease to the inability of the patient's neutrophils to seek and clear infections due to an inherited deficiency in leukocyte adhesion caused by the loss of a plasma membrane glycoprotein complex now known as CD11/CD18 or β2 integrins. I followed this work by determining the 3-dimensional structures of integrins. These studies provided the foundation for understanding the unique properties of integrins in mediating bidirectional cell adhesion signaling and enabled a structure-guided design of compounds to dial down overactive integrins in common disorders, including thromboinflammatory and autoimmune diseases.
先天性免疫细胞在检测、搜寻和清除入侵病原体方面发挥着至关重要的作用,从而确保宿主存活。然而,这些细胞功能丧失或它们对组织损伤的过度活跃反应往往会导致严重疾病。因此,从基础层面了解这些细胞在健康和疾病中的功能至关重要。朝着这一目标迈出的重要一步来自我在20世纪70年代后期进行的研究,这些研究调查了一名儿科患者危及生命的细菌感染的原因。这项工作使我们将这种疾病追溯到患者的中性粒细胞由于一种遗传性白细胞黏附缺陷而无法搜寻和清除感染,这种缺陷是由一种现在被称为CD11/CD18或β2整合素的质膜糖蛋白复合物缺失所致。在这项工作之后,我确定了整合素的三维结构。这些研究为理解整合素在介导双向细胞黏附信号传导中的独特特性奠定了基础,并使得能够进行结构导向的化合物设计,以降低常见疾病(包括血栓炎症性疾病和自身免疫性疾病)中过度活跃的整合素的活性。