Pirofski L, Horwitz M S, Scharff M D, Factor S M
Department of Medicine, Albert Einstein College of Medicine, Bronx, NY 10461.
Proc Natl Acad Sci U S A. 1991 May 15;88(10):4358-62. doi: 10.1073/pnas.88.10.4358.
Murine adenovirus type 1 (MAV-1) infection of CB-17 SCID mice (which are homozygous for the severe combined immunodeficiency mutation) induces hepatic histopathologic and ultrastructural features that are strikingly similar to human Reye syndrome. Gross pathologic examination of MAV-1-infected mice revealed only pale yellow liver tissue. Histopathologic studies of tissue from MAV-1-infected mice revealed diffuse hepatic injury manifested by microvesicular fatty degenerative changes of hepatocytes and electron microscopic evidence of focal mitochondrial swelling with disruption of cristae and depletion of glycogen. Serum aminotransferase activities increased markedly in the infected animals; however, plasma ammonia levels were not elevated at the times assayed. Although all mice infected with MAV-1 died, neutralizing anti-MAV-1 monoclonal antibodies provided a dose-dependent delay in the appearance of clinical disease and hepatic histopathologic findings. Other findings included rare viral inclusions with only minimal inflammation in spleen, adrenal, and liver of infected mice. Our findings indicate that MAV-1 infection of SCID mice may provide important insights into the pathogenesis of the hepatic lesions of Reye syndrome.
1型鼠腺病毒(MAV-1)感染CB-17 SCID小鼠(严重联合免疫缺陷突变的纯合子)会诱发肝脏组织病理学和超微结构特征,这些特征与人瑞氏综合征极为相似。对感染MAV-1的小鼠进行大体病理检查,仅发现淡黄色肝组织。对感染MAV-1小鼠的组织进行组织病理学研究,发现弥漫性肝损伤,表现为肝细胞微泡性脂肪变性改变,以及电镜下局灶性线粒体肿胀、嵴断裂和糖原耗竭的证据。感染动物的血清转氨酶活性显著升高;然而,在所检测的时间点血浆氨水平并未升高。尽管所有感染MAV-1的小鼠均死亡,但中和抗MAV-1单克隆抗体可剂量依赖性地延迟临床疾病和肝脏组织病理学表现的出现。其他发现包括感染小鼠的脾脏、肾上腺和肝脏中罕见的病毒包涵体,炎症轻微。我们的研究结果表明,SCID小鼠感染MAV-1可能为深入了解瑞氏综合征肝脏病变的发病机制提供重要线索。