Immunocytochemical examination of Reye's syndrome

Reye综合征的病因和发病机制尚不清楚。特征性表现为线粒体损伤，线粒体酶活性降低，内脏脂肪堆积。Woodfin等人。报道称，雷氏综合征患者肝脏中的线粒体酶被释放到胞浆中。然而，线粒体酶在Reye综合征中的定位尚未被研究。在本研究中。我们研究了鸟氨酸转氨甲基酶(OTC)的定位。用光镜和电子显微镜对雷氏综合征和雷氏样综合征患者的肝脏进行免疫组织化学染色，作为线粒体酶的标志。在Reye样综合征患者中，部分患者被诊断为OTC缺乏。光镜下，将小组织切片包埋于EPON中，用抗牛OTC兔Ig G和免疫酶技术进行染色。在电子显微镜下，肝组织切片用Lowicryl K4M包埋，用抗牛OTC兔抗体和蛋白A-金标记法标记。男性OTC缺乏症患者肝细胞未见OTC染色，女性OTC缺乏症患者肝细胞呈由OTC阳性细胞和OTC阴性细胞组成的细胞嵌合体。提示肝OTC免疫细胞化学检查有助于OTC缺乏症的诊断。在Reye综合征肝细胞的电子显微镜观察中，胞浆中未检测到代表OTC抗原性的金颗粒，而局限于线粒体基质中。这些结果表明，在雷氏综合征中，肝脏线粒体酶不是从线粒体释放到胞浆中，而是局限于线粒体中。

The etiology and pathogenesis of Reye's syndrome remains obsure. The characteristic findings are mitochondrial damage, decrease of the activity of mitochondrial enzymes and fatty accumulation in the viscera. Woodfin et al. reported that mitochondrial enzymes are released from the mitochondria to the cytosol in the liver of Reye's syndrome. However the localization of the mitochondrial enzyme in Reye's syndrome has not been investigated. In the present study. we investigated the localization of ornithine transcarbamylase (OTC). as a marker of mitochondrial enzymes, in the liver of the patients with Reye's syndrome or Reye like syndrome immunochemically using light and electron microscope. In the patients with Reye like syndrome, some were diagnosed as OTC deficiencey. For the light microscopy, small tissue slices of the liver were embedded in Epon and stained by anti-bovine OTC rabbit IgG and the immunoenzyme technique. For the electron microscopy, slices of liver tissues were embedded in Lowicryl K4M and labeled by anti-bovine OTC rabbit IgG and the protein A-gold technique. The liver cells of the male patients with OTC deficiency were not stained for OTC, while those of the female patients with OTC deficiency showed a cellular mosaic consisting of OTC-positive and OTC-negative cells. These results indicate that immunocytochemical examination of liver OTC is useful for diagnosis of OTC deficiency. In the electron microscopic observation of the liver cells of Reye's syndrome, gold particles representing the anti-genic sites for OTC were not detected in the cytosol, but confined in the mitochondrial matrix. Those results show that liver mitochondrial enzymes are not released from mitochondria to the cytosol, but confine in the mitochondria in Reye's syndrome.