Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients
Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients
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DOI：
10.1007/s00401-008-0425-8
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发表时间：
2008-11-01
影响因子：
12.7
通讯作者：
Kitamoto, Tetsuyuki
中科院分区：
文献类型：
--
作者：
Kobayashi, Atsushi;Arima, Kunimasa;Kitamoto, Tetsuyuki
PrP基因第129位密码子甲硫氨酸纯合子和PrP 1型异常亚型(sCJD-MM1)的散发性CreutzFeldt-Jakob病患者脑内PrP的斑块型沉积是极其罕见的。在这里，我们报告了三名sCJD-MM1患者，他们在大脑和小脑白质中显示出明显的PrP阳性淀粉样斑块。3例患者均表现为长期(2年)的临床病程
Plaque-type deposition of prion protein (PrP) in the brain has been extremely rare in sporadic Creutzfeldt-Jakob disease patients with methionine homozygosity at polymorphic codon 129 of the PrP gene and type 1 abnormal isoform of PrP (sCJD-MM1). Here we report three sCJD-MM1 patients who showed prominent PrP-positive amyloid plaques in the cerebral and cerebellar white matter. All three patients showed clinical courses of long duration (2 years