Rapid Detection of Intracellular p47phox and p67phox by Flow Cytometry; Useful Screening Tests for Chronic Granulomatous Disease.
Rapid Detection of Intracellular p47phox and p67phox by Flow Cytometry; Useful Screening Tests for Chronic Granulomatous Disease.
复制标题
流式细胞仪快速检测细胞内p47phox和p67phox；
DOI：
10.1007/s10875-012-9859-9
复制
发表时间：
2013
期刊：
影响因子：
--
通讯作者：
Wada T
中科院分区：
文献类型：
--
作者：
Yamamoto A;Morio T;Kumaki E;Yamazaki H;Iwai H;Kubota T;Miyasaka N;Kohsaka H.;Wada T
慢性肉芽肿病（CGD）是由 NADPH 氧化酶缺陷引起的。 CGD的诊断可以通过分析NADPH氧化酶活性来进行，但是在进行突变分析之前需要鉴定CGD亚组。与胞质成分 p47phox 和 p67phox 不同，膜结合亚基 gp91phox 和 p22phox 可以通过流式细胞术快速分析。我们评估了使用特异性单克隆抗体流式细胞术检测 2 名 p47phox 缺陷患者和 7 名 p67phox 缺陷患者的 p47phox 和 p67phox 的可行性。与之前的观察结果一致，正常对照中 p47phox 和 p67phox 在吞噬细胞和 B 细胞中表达，但在 T 细胞或自然杀伤细胞中不表达。相比之下，p47phox 和 p67phox 缺乏的患者的 p47phox 和 p67phox 水平分别显着降低。这些技术将有助于快速评估胞质成分 p47phox 和 p67phox 的表达，并且代表了 CGD 的重要二级筛选测试。
Chronic granulomatous disease (CGD) is caused by defects of NADPH oxidase. The diagnosis of CGD can be made by analysis of NADPH oxidase activity, however, identification of the CGD subgroups is required before performing mutation analysis. The membrane-bound subunits, gp91phox and p22phox, can be quickly analyzed by flow cytometry, unlike the cytosolic components, p47phox and p67phox. We evaluated the feasibility of flow cytometric detection of p47phox and p67phox with specific monoclonal antibodies in two patients with p47phox deficiency and 7 patients with p67phox deficiency. Consistent with previous observations, p47phox and p67phox were expressed in phagocytes and B cells, but not in T or natural killer cells, from normal controls. In contrast, patients with p47phox and p67phox deficiency showed markedly reduced levels of p47phox and p67phox, respectively. These techniques will be useful to rapidly assess the expression of the cytosolic components, p47phox and p67phox, and represents important secondary screening tests for CGD.