Two cytosolic neutrophil oxidase components absent in autosomal chronic granulomatous disease.
Two cytosolic neutrophil oxidase components absent in autosomal chronic granulomatous disease.
复制标题
常染色体慢性肉芽肿病中不存在两种胞质中性粒细胞氧化酶成分。
DOI：
10.1126/science.2848318
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发表时间：
1988
期刊：
影响因子：
--
通讯作者：
Clark,RA
中科院分区：
文献类型：
--
作者：
Volpp,BD;Nauseef,WM;Clark,RA
中性粒细胞通过氧化酶产生的氧自由基杀死微生物，氧化酶使用还原形式的烟酰胺腺嘌呤二核苷酸磷酸（NADPH）作为底物。该系统需要膜和胞质组分，并且在慢性肉芽肿性疾病患者中是有缺陷的。从鸟苷三磷酸-琼脂糖中洗脱出能够激活潜伏膜氧化酶的胞质复合物，并用于提高识别47-和67-千道尔顿蛋白的多克隆抗血清。这些蛋白质局限于髓样细胞的胞质溶胶。这两种蛋白质与NADPH氧化酶激活能力时，中性粒细胞胞质溶胶纯化核苷酸亲和矩阵或分子大小列。两种不同形式的常染色体慢性肉芽肿病患者的中性粒细胞缺乏47或67千道尔顿蛋白。
Neutrophils kill microorganisms with oxygen radicals generated by an oxidase that uses the reduced form of nicotinamide adenine dinucleotide phosphate (NADPH) as substrate. This system requires both membrane and cytosolic components and is defective in patients with chronic granulomatous disease. A cytosolic complex capable of activating latent membrane oxidase was eluted from guanosine triphosphate-agarose and was used to raise polyclonal antiserum that recognized 47- and 67-kilodalton proteins. These proteins were restricted to the cytosol of myeloid cells. Both proteins were associated with NADPH oxidase-activating capacity when neutrophil cytosol was purified on nucleotide affinity matrices or molecular sizing columns. Neutrophils from patients with two different forms of autosomal chronic granulomatous disease lacked either the 47- or 67-kilodalton protein.