Breton-Gorius J, Coquin Y, Guichard J
Lab Invest. 1978 Jan;38(1):21-31.
The neutrophils and monocytes of two patients with hereditary myeloperoxidase (MPO) deficiency lacked MPO activity as determined by light and electron microscopic cytochemical staining. With a technique employing neutral 3,3'-diaminobenzidine, azurophils of precursor and mature neutrophils were devoid of MPO whereas eosinophil, basophil, and platelet peroxidases exhibited normal activity. After incubation in alkaline DAB medium, which stains catalase, some small granules were strongly reactive in both immature and mature neutrophils and monocytes. These catalase-containing granules were distinct from all other categories of granules. Their number decreased with maturation. In the presence of cyanide or aminotriazole, peroxidatic activity could also be detected in ellipsoid azurophils, although large spherical granules remained unreactive. This peroxidatic activity is apparently not due to MPO inasmuch as it has been demonstrated that this protein is not synthesized in these patients. Thus, the significance of the last finding is unclear but suggests a heterogeneity of azurophil content. In contrast to human MPO-deficient cells, chicken heterophils naturally devoid of peroxidase are unable to produce hydrogen peroxide upon phagocytosis and were also devoid of catalase-containing particles. This observation suggests that catalase is involved in the control of the intracellular level of hydrogen peroxide in human cells.
通过光镜和电镜细胞化学染色测定,两名遗传性髓过氧化物酶(MPO)缺乏症患者的中性粒细胞和单核细胞缺乏MPO活性。使用中性3,3'-二氨基联苯胺的技术显示,早幼粒细胞和成熟中性粒细胞的嗜天青颗粒缺乏MPO,而嗜酸性粒细胞、嗜碱性粒细胞和血小板过氧化物酶表现出正常活性。在用于染色过氧化氢酶的碱性DAB培养基中孵育后,一些小颗粒在未成熟和成熟的中性粒细胞及单核细胞中均呈强反应性。这些含过氧化氢酶的颗粒与所有其他类型的颗粒不同。其数量随细胞成熟而减少。在氰化物或氨基三唑存在的情况下,椭圆形嗜天青颗粒中也可检测到过氧化物活性,尽管大的球形颗粒仍无反应。这种过氧化物活性显然不是由MPO引起的,因为已证明这些患者不合成这种蛋白质。因此,最后这一发现的意义尚不清楚,但提示嗜天青颗粒成分存在异质性。与人类MPO缺乏细胞不同,天然缺乏过氧化物酶的鸡异嗜性粒细胞在吞噬时不能产生过氧化氢,并且也不含含过氧化氢酶的颗粒。这一观察结果表明,过氧化氢酶参与了人类细胞内过氧化氢水平的调控。