Distinct origins of dura mater graft-associated Creutzfeldt-Jakob disease: past and future problems.
Distinct origins of dura mater graft-associated Creutzfeldt-Jakob disease: past and future problems.
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DOI：
10.1186/2051-5960-2-32
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发表时间：
2014-03-31
影响因子：
7.1
通讯作者：
Kitamoto T
中科院分区：
文献类型：
--
作者：
Kobayashi A;Matsuura Y;Mohri S;Kitamoto T
硬脑膜移植相关的克雅氏病 (dCJD) 可分为两个亚组，表现出不同的临床和神经病理学特征，其中大多数为非斑块型 dCJD (np-dCJD)，少数为斑块型 dCJD (p-dCJD)。 dCJD 的两种不同表型被认为与 PRNP 基因多态性密码子 129 的基因型（蛋氨酸，M 或缬氨酸，V）或大脑中异常朊病毒蛋白（PrPSc）的类型（1 型或 2 型）无关，而这些是散发性克雅病（sCJD）临床病理表型的主要决定因素。 dCJD 中存在两个不同亚组的原因仍然难以捉摸。 dCJD发病机制的最新研究进展表明，dCJD有两个不同的亚型是由sCJD不同的PrPSc毒株感染引起的，即由sCJD-MM1/MV1感染引起的np-dCJD和由sCJD-VV2或-MV2感染引起的p-dCJD。这些研究还揭示了以前未被认识到的问题如下：（i）未来p-dCJD患者数量可能会增加，（ii）dCJD，特别是p-dCJD继发感染的潜在风险可能相当大，以及（iii）目前针对p-dCJD的PrPSc去污程序的有效性尚不确定。为了预防p-dCJD继发感染，建立有效的净化程序是一个紧迫的问题。在这篇综述中，我们总结了围绕 dCJD 的过去和未来的问题。
Dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) can be divided into two subgroups that exhibit distinct clinical and neuropathological features, with the majority represented by a non-plaque-type of dCJD (np-dCJD) and the minority by a plaque-type of dCJD (p-dCJD). The two distinct phenotypes of dCJD had been considered to be unrelated to the genotype (methionine, M or valine, V) at polymorphic codon 129 of the PRNP gene or type (type 1 or type 2) of abnormal isoform of prion protein (PrPSc) in the brain, while these are major determinants of clinicopathological phenotypes of sporadic CJD (sCJD). The reason for the existence of two distinct subgroups in dCJD had remained elusive. Recent progress in research of the pathogenesis of dCJD has revealed that two distinct subgroups of dCJD are caused by infection with different PrPSc strains from sCJD, i.e., np-dCJD caused by infection with sCJD-MM1/MV1, and p-dCJD caused by infection with sCJD-VV2 or -MV2. These studies have also revealed previously unrecognized problems as follows: (i) the numbers of p-dCJD patients may increase in the future, (ii) the potential risks of secondary infection from dCJD, particularly from p-dCJD, may be considerable, and (iii) the effectiveness of the current PrPSc decontamination procedures against the PrPSc from p-dCJD is uncertain. To prevent secondary infection from p-dCJD, the establishment of effective decontamination procedures is an urgent issue. In this review, we summarize the past and future problems surrounding dCJD.
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影响因子：
7.1
作者：
Kobayashi A;Iwasaki Y;Otsuka H;Yamada M;Yoshida M;Matsuura Y;Mohri S;Kitamoto T
通讯作者：
Kitamoto T
影响因子：
158.5
作者：
BROWN, P;GIBBS, CJ;GAJDUSEK, DC
通讯作者：
GAJDUSEK, DC
影响因子：
11.8
作者：
Brown P;Brandel JP;Sato T;Nakamura Y;MacKenzie J;Will RG;Ladogana A;Pocchiari M;Leschek EW;Schonberger LB
通讯作者：
Schonberger LB
影响因子：
--
作者：
Shimizu, S;Hoshi, K;Kitamoto, T
通讯作者：
Kitamoto, T
影响因子：
4.8
作者：
Kobayashi, Atsushi;Asano, Masahiro;Kitamoto, Tetsuyuki
通讯作者：
Kitamoto, Tetsuyuki