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患有慢性消耗病的落基山麋鹿大脑中的纤维含有羊瘙痒病淀粉样蛋白。

Fibrils in brain of Rocky Mountain elk with chronic wasting disease contain scrapie amyloid.

作者信息

Guiroy D C, Williams E S, Song K J, Yanagihara R, Gajdusek D C

机构信息

Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.

出版信息

Acta Neuropathol. 1993;86(1):77-80. doi: 10.1007/BF00454902.

Abstract

Chronic wasting disease (CWD), a progressive, fatal neurological disorder of captive mule deer and Rocky Mountain elk, is characterized neuropathologically by spongiform change in the neuropil, intraneuronal vacuolation and astrocytic hypertrophy and hyperplasia. Recently, scrapie amyloid-immunoreactive plaques have been demonstrated in brain tissues of CWD-affected captive mule deer, Rocky Mountain elk and hybrids of captive mule deer and white-tailed deer. We now report on the presence of abnormal fibrils isolated from brain tissues of Rocky Mountain elk using negative-stain electron microscopy. These fibrils resemble those found in scrapie-infected hamster brain. Furthermore, protein bands with relative molecular masses of 26 to 30 kilodaltons were shown to be immunoreactive to antibodies raised against scrapie amyloid by Western immunoblotting. Immuno-dot blot showed similar reactivity. Our data support the clinical and pathological diagnosis of the disease and provide further evidence that CWD belongs to the subacute spongiform encephalopathies.

摘要

慢性消耗病(CWD)是圈养骡鹿和落基山麋鹿的一种进行性、致命性神经疾病,其神经病理学特征为神经纤维网海绵状改变、神经元内空泡形成以及星形胶质细胞肥大和增生。最近,在受CWD影响的圈养骡鹿、落基山麋鹿以及圈养骡鹿与白尾鹿的杂交种的脑组织中发现了瘙痒病淀粉样免疫反应性斑块。我们现在报告利用负染电子显微镜从落基山麋鹿脑组织中分离出异常纤维的情况。这些纤维类似于在感染瘙痒病的仓鼠脑中发现的纤维。此外,通过蛋白质免疫印迹法显示,相对分子质量为26至30千道尔顿的蛋白条带对针对瘙痒病淀粉样蛋白产生的抗体具有免疫反应性。免疫斑点印迹显示出类似的反应性。我们的数据支持该疾病的临床和病理诊断,并提供了进一步证据表明慢性消耗病属于亚急性海绵状脑病。

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