慢性肉芽肿病的p47phox小鼠基因敲除模型。
The p47phox mouse knock-out model of chronic granulomatous disease.
作者信息
Jackson S H, Gallin J I, Holland S M
机构信息
Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892-1886, USA.
出版信息
J Exp Med. 1995 Sep 1;182(3):751-8. doi: 10.1084/jem.182.3.751.
Abstract
Chronic granulomatous disease (CGD) is caused by a congenital defect in phagocyte reduced nicotinamide dinucleotide phosphate (NADPH) oxidase production of superoxide and related species. It is characterized by recurrent life-threatening bacterial and fungal infections and tissue granuloma formation. We have created a mouse model of CGD by targeted disruption of p47phox, one of the genes in which mutations cause human CGD. Identical to the case in human CGD, leukocytes from p47phox-/- mice produced no superoxide and killed staphylococci ineffectively. p47phox-/- mice developed lethal infections and granulomatous inflammation similar to those encountered in human CGD patients. This model mirrors human CGD and confirms a critical role for the phagocyte NADPH oxidase in mammalian host defense.
摘要
慢性肉芽肿病（CGD）是由吞噬细胞还原型烟酰胺腺嘌呤二核苷酸磷酸（NADPH）氧化酶产生超氧化物及相关物质的先天性缺陷所引起。其特征为反复发生危及生命的细菌和真菌感染以及组织肉芽肿形成。我们通过靶向破坏p47phox基因（该基因突变会导致人类CGD）创建了一种CGD小鼠模型。与人类CGD情况相同，来自p47phox基因敲除小鼠的白细胞不产生超氧化物，并且杀灭葡萄球菌的能力低下。p47phox基因敲除小鼠发生了致死性感染和肉芽肿性炎症，类似于人类CGD患者所遭遇的情况。该模型反映了人类CGD，并证实了吞噬细胞NADPH氧化酶在哺乳动物宿主防御中的关键作用。
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