Chronic granulomatous disease (CGD) is a phagocytic disorder characterized by a defective production of reactive oxygen species (ROSs). Although infections and granuloma formation are the most common manifestations in CGD patients, a significant number of patients experienced autoimmunity and inflammatory diseases suggesting that adaptive immune abnormalities might be involved.

Here we investigated T-cell compartment and showed that CGD patients had a skewed TCRV-beta distribution in CD8+ T cells, particularly in older patients, and a reduced proliferative responses toward mitogens compared to healthy donors (HD). Afterwards we studied the role of gp91phox protein in causing these alterations and demonstrated that human T cells do not express gp91phox and TCR-stimulated ROS generation is gp91phox-NADPH oxidase independent. Finally, we proved that the NADPH oxidase is not active in the T cell compartment even when forcing gp91phox expression transducing T cells from X-CGD and HD with a SIN lentiviral vector (LVV) encoding the gp91phox cDNA.

慢性肉芽肿性疾病（CGD）是吞噬性疾病，其特征在于活性氧（ROSs）产生缺陷。尽管感染和肉芽肿形成是CGD患者中最常见的表现，但是许多患者经历了自身免疫和炎症性疾病，提示可能涉及了适应性免疫异常。

在这里，我们研究了T细胞区室，结果表明CGD患者在CD8 + T细胞中，尤其是在老年患者中，TCRV-beta分布偏斜，与健康供体（HD）相比，对有丝分裂原的增殖反应降低。之后，我们研究了gp91phox蛋白在引起这些改变中的作用，并证明人T细胞不表达gp91phox，而TCR刺激的ROS生成是gp91phox-NADPH氧化酶的独立反应。最后，我们证明了即使使用编码gp91phox cDNA的SIN慢病毒载体（LVV）强迫从X-CGD和HD转导gp91phox表达的T细胞时，NADPH氧化酶在T细胞区室中也没有活性。