Spielberg S P, Boxer L A, Oliver J M, Allen J M, Schulman J D
Br J Haematol. 1979 Jun;42(2):215-23. doi: 10.1111/j.1365-2141.1979.tb01126.x.
Several episodes of neutropenia were observed in a child with glutathione synthetase deficiency (5-oxoprolinuria). Studies of the patient's glutathione-deficient neutrophils were undertaken to examine the responses of the cells to oxidative stress associated with phagocytosis. The patient's neutrophils contained 10--20% of normal glutathione content. Circulating neutrophils in infection-free periods appeared less mature than normal by morphologic criteria, suggesting increased cell turnover. The cells ingested particles, responded to chemotactic stimuli, and oxidized 1-14C glucose normally. However, following ingestion of particles, the cells accumulated excess hydrogen peroxide compared with normal cells, and showed impaired protein iodination and bacterial killing. Electron micrographs revealed damage to microtubules and membranous structures in the patient's neutrophils during phagocytosis. The level of glutathione in the cells appears inadequate to protect against peroxide generated during normal cell function, and the cells are thus damaged and rendered less effective in bacterial killing. The data provide evidence for a protective role of glutathione in normal neutrophil function.
在一名患有谷胱甘肽合成酶缺乏症（5-氧脯氨酸尿症）的儿童中观察到了几次中性粒细胞减少症。对该患者缺乏谷胱甘肽的中性粒细胞进行了研究，以检查细胞对与吞噬作用相关的氧化应激的反应。患者的中性粒细胞中谷胱甘肽含量为正常含量的10%-20%。根据形态学标准，在无感染期循环中的中性粒细胞看起来比正常细胞成熟度更低，提示细胞更新增加。这些细胞摄取颗粒，对趋化刺激有反应，并能正常氧化1-14C葡萄糖。然而，与正常细胞相比，在摄取颗粒后，这些细胞积累了过量的过氧化氢，并且表现出蛋白质碘化和细菌杀伤受损。电子显微镜照片显示，在吞噬过程中患者的中性粒细胞中的微管和膜结构受到损伤。细胞内的谷胱甘肽水平似乎不足以保护细胞免受正常细胞功能过程中产生的过氧化物的影响，因此细胞受到损伤，在细菌杀伤方面变得效率更低。这些数据为谷胱甘肽在正常中性粒细胞功能中的保护作用提供了证据。
