Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients

被引:0
作者
Atsushi Kobayashi
Kunimasa Arima
Masafumi Ogawa
Miho Murata
Takahiro Fukuda
Tetsuyuki Kitamoto
机构
[1] Tohoku University Graduate School of Medicine,Division of CJD Science and Technology, Department of Prion Research
[2] National Center of Neurology and Psychiatry Musashi Hospital,Department of Laboratory Medicine
[3] National Center of Neurology and Psychiatry Musashi Hospital,Department of Neurology
[4] The Jikei University School of Medicine,Division of Neuropathology, Department of Neuroscience, Research Center for Medical Sciences
来源
Acta Neuropathologica | 2008年 / 116卷
关键词
Creutzfeldt-Jakob disease; Prion protein; Amyloid plaque; White matter;
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中图分类号
学科分类号
摘要
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 ≤), particularly at the end-stage. The white matter of these patients was severely damaged because of the prolonged disease duration. Furthermore, Alzheimer’s amyloid precursor protein, which accumulates within the axonal swellings under pathological conditions, co-accumulated with the PrP-amyloid plaques. These findings suggest that the axonal damage reflecting the prolonged disease duration causes the deposition of PrP-amyloid plaques in the white matter. The present study shows that PrP-amyloid plaques can occur in the white matter of sCJD-MM1 cases.
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页码:561 / 566
页数:5
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