Stimulated neutrophils from patients with autosomal recessive chronic granulomatous disease fail to phosphorylate a Mr-44,000 protein
Stimulated neutrophils from patients with autosomal recessive chronic granulomatous disease fail to phosphorylate a Mr-44,000 protein
复制标题
来自常染色体隐性慢性肉芽肿病患者的刺激中性粒细胞未能磷酸化 Mr-44,000 蛋白
DOI：
10.1038/316547a0
复制
发表时间：
1985
期刊：
影响因子：
64.8
通讯作者：
M. Barrowman
中科院分区：
文献类型：
--
作者：
A. Segal;P. Heyworth;S. Cockcroft;M. Barrowman
吞噬中性粒细胞、单核细胞、巨噬细胞和嗜酸性粒细胞会产生一阵非线粒体呼吸，这对于杀死和消化微生物非常重要。有关氧化酶系统的大部分信息都来自对慢性肉芽肿病 (CGD) 患者的研究，慢性肉芽肿病是一种由于完全缺乏这种刺激性呼吸活动而导致过度感染的综合征。氧化酶活性的基础是电子传递链，其唯一确定的成分是非常不寻常的 b 型细胞色素 (b−245)（参考文献 2）。 CGD X连锁亚组的分子缺陷是缺乏这种细胞色素b−245，然而，在具有常染色体隐性遗传模式的受试者中，这种细胞色素b−245似乎是正常的3。为了确定后一组中电子传递链近端组件的激活异常或缺失或故障，我们检查了用佛波醇肉豆蔻酸酯乙酸酯激活氧化酶后中性粒细胞中的蛋白质磷酸化。研究的所有四名患者都表现出选择性缺乏相对分子质量 (Mr) 44,000 (44K) 的蛋白质的增强磷酸化，这种现象在正常受试者和两名具有 X 连锁遗传的 CGD 患者中观察到。因此，该分子可能是氧化酶的重要功能成分。
Phagocytosing neutrophils, monocytes, macrophages and eosino-phils produce a burst of non-mitochondrial respiration that is important for the killing and digestion of microbes. Much of the information about the oxidase system involved comes from studies on patients with chronic granulomatous disease (CGD), a syndrome in which an undue predisposition to infection results from complete absence of this burst of stimulated respiratory activity1. The basis of the oxidase activity is an electron transport chain, the only established component of which is a very unusual b-type cyto-chrome (b−245) (ref. 2). The molecular defect in the X-linked subgroup of CGD is the absence of this cytochrome b−245, which, however, appears to be normal in those subjects with the autosomal recessive mode of inheritance3. In an attempt to identify an abnormality of activation, or an absence or malfunction of a proximal component of the electron transport chain in this latter group, we examined protein phosphorylation in neutrophils after activation of the oxidase with phorbol myristate acetate. All four of the patients studied demonstrated a selective lack of the enhanced phosphorylation of a protein of relative molecular mass (Mr) 44,000 (44K) that was observed in normal subjects and in two CGD patients with an X-linked inheritance. This molecule, therefore, could be an important functional component of the oxidase.
静息和激活的人中性粒细胞的内源性蛋白质磷酸化。
影响因子：
20.3
作者：
Andrews,PC;Babior,BM
通讯作者：
Babior,BM
人多形核白细胞产生超氧化物的 NADPH 氧化酶的辅因子需求特征。
影响因子：
2.9
作者：
Light,DR;Walsh,C;O'Callaghan,AM;Goetzl,EJ;Tauber,AI
通讯作者：
Tauber,AI
静止和激活的人类中性粒细胞对胞质蛋白的磷酸化。
影响因子：
20.3
作者：
Andrews,PC;Babior,BM
通讯作者：
Babior,BM