CREUTZFELDT-JAKOB DISEASE WITH AMYLOID ANGIOPATHY - DIAGNOSIS BY IMMUNOLOGICAL ANALYSES AND TRANSMISSION EXPERIMENTS

CREUTZFELDT-JAKOB DISEASE WITH AMYLOID ANGIOPATHY - DIAGNOSIS BY IMMUNOLOGICAL ANALYSES AND TRANSMISSION EXPERIMENTS
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DOI:
10.1007/bf00310037
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发表时间:
1992-04-01
影响因子:
12.7
通讯作者:
PEIFFER, J
PEIFFER, J
中科院分区:
医学1区
文献类型:
--
作者:
TATEISHI, J;KITAMOTO, T;PEIFFER, J

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摘要1例73岁男性克雅氏病(CJD)由于广泛的淀粉样血管病,缺乏任何严重的海绵状改变,难以作出明确的病理诊断。抗PrP抗体免疫染色显示,组织切片经水解性高压灭菌处理后,灰质内有细小颗粒状沉积。Western blotting也发现了PrP的异常亚型,但PrP基因分析没有显示任何异常。一次传播实验重复三次,经过较长时间的潜伏期后,少数小鼠出现海绵状脑病。
It was difficult to make a definite pathological diagnosis in a 73-year-old man with Creutzfeldt-Jakob disease (CJD) due to extensive amyloid angiopathy which lacked any severe spongiform changes. Immunostaining using anti-prion protein (PrP) antibody revealed fine granular deposits in the gray matter, after hydrolytic autoclaving pretreatment on tissue sections. Western blotting also revealed an abnormal isoform of PrP, but PrP gene analysis did not show any abnormalities. The primary transmission experiments were repeated three times and induced spongiform encephalopathy in a few mice after a long incubation period.