检索结果-内蒙古大学图书馆

Stealth virus epidemic in the Mohave Valley: Severe vacuolating encephalopathy in a child presenting with a behavioral disorder

引用

EXPERIMENTAL AND MOLECULAR PATHOLOGY 1999年第1期66卷 19-30页

作者： Martin, WJ Anderson, D Ctr Complex Infect Dis Rosemead CA 91770 USA

An infectious illness, attributed to atypically structured cytopathic "stealth" viruses, occurred in 1996 in the Mohave Valley region of the United States. A stealth virus-infected child from this region has developed a severe noninflammatory, vacuolating (spongiform) encephalopathy. The illness initially presented as a behavioral problem without overt neurological signs. Extensive investigations, including repeated magnetic resonance imaging, two brain biopsies, and stealth virus cultures, have helped define the disease process occurring in this child. Significant clinical benefit with apparent retardation of disease progression occurred during a 6-week course of ganciclovir therapy. The potential contributing role of stealth virus infections in children presenting with behavioral problems needs to be addressed. (C) 1999 Academic Press.

关键词：抗病毒药/治疗应用脑/病理学脑/超微结构加利福尼亚/流行病学更昔洛韦/治疗应用磁共振成像精神障碍/病因学精神障碍/病毒学朊病毒病/药物疗法朊病毒病/流行病学朊病毒病/心理学朊病毒病/病毒学回顾性研究空泡/病理学空泡/超微结构病毒性疾病/诊断病毒性疾病/药物疗法病毒性疾病/流行病学病毒性疾病/心理学儿童人类男(雄)性

来源：评论

学校读者我要写书评

暂无评论

Quantifying the dynamics of prion infection: a bifurcation analysis of Laurent's model

引用

JOURNAL OF THEORETICAL BIOLOGY 2000年第2期205卷 283-296页

作者： Porcher, E Gatto, M Politecn Milan Dipartimento Elettron & Informaz I-20133 Milan Italy Politecn Milan Ctr Studio Ingn Biomed CNR I-20133 Milan Italy Univ Paris Sud Lab Ecol Systemat & Evolut F-91405 Orsay France

Laurent(1996a, Medecine/sciences 12, 774-785;1996b, Biochem. J. 318, 35-39;1998, Bio-phys. Chem. 72, 211-222) proposed a model for the dynamics of diseases of the central nervous system caused by prions. It is based on the protein-only hypothesis (Prusiner ct al., 1981, Proc. Natl. Acad. Sci. U.S.A. 78, 6675-6679), which assumes that infection can be spread by particular proteins (prions) that can exist in two forms that share the same sequence, but have a different structure. The normal form is harmless, while the infectious isoform of the prion protein catalyses a transconformation from the native isoform to itself within a specialized compartment of the brain cells. This paper systematically explores the model behavior with the aim of quantifying the fundamental parameters characterizing the dynamics of prion infection. To this end we use data from the literature to fix orders of magnitude for the rates of synthesis and degradation of the native form of prion protein and for the shape of the autocatalytic function. The dynamical behavior is classified with respect to two unknown parameters (bifurcation analysis): the rate of spontaneous transconformation and the rate of output of the infectious isoform from the specialized compartment. We thus find that the bistability properties evidenced by Laurent are confined to a certain range of parameters and that permanent oscillations of the two isoforms concentrations are possible. The bifurcation analysis allows us to estimate approximate ranges for the values of the two unknown parameters and consequently to derive incubation times and compare them with actual data for hamster. Also, our study predicts that the output rate of the infectious isoform is relatively insensitive to variations of model parameters. (C) 2000 Academic Press.

关键词：脑/代谢仓鼠亚科模型生物学模型统计学朊病毒病/流行病学朊病毒病/病因学朊病毒病/代谢朊病毒/代谢蛋白质亚型/代谢动物人类

来源：评论

学校读者我要写书评

暂无评论

Is the pathogen of prion disease a microbial protein?

引用

MEDICAL HYPOTHESES 1999年第2期53卷 91-102页

作者： Füzi, M Budapest Inst Natl Publ Hlth & Med Officer Serv H-1138 Budapest Hungary

Though considerable circumstantial evidence suggests that the pathogen of prion disease is proteinaceous, it has not yet been conclusively identified. Epidemiological observations indicate that a microbial vector is responsible for the transmission of natural prion disease in sheep and goats and that the real causative agent may correspond to a structural protein of that microorganism. The microbial protein should resemble prion protein (PrP) and may replicate itself in the host by using mammalian DNA. A similar phenomenon was already described with a protein antigen of the ameba Naegleria gruberi (1-4). The various serotypes of the microbial protein may account for the existence of scrapie strains. It is proposed that many microbial proteins may be capable of replicating themselves in mammalian cells eliciting and sustaining thereby degenerative and/or autoimmune reactions subsequent to infections with microorganisms.

关键词：动物基因修饰克-亚综合征/流行病学克-亚综合征/病因学克-亚综合征/传播鹿疾病媒介 Gerstmann-Straussler-Scheinker病/流行病学 Gerstmann-Straussler-Scheinker病/病因学 Gerstmann-Straussler-Scheinker病/传播山羊疾病/病因学山羊疾病/传播山羊库鲁病/流行病学库鲁病/病因学库鲁病/传播朊病毒病/流行病学朊病毒病/病因学朊病毒病/传播朊病毒/致病力羊瘙痒症/流行病学羊瘙痒症/病因学羊瘙痒症/传播绵羊动物

来源：评论

学校读者我要写书评

暂无评论

Molecular genetics of human prion diseases in Germany

引用

HUMAN GENETICS 1999年第3期105卷 244-252页

作者： Windl, O Giese, A Schulz-Schaeffer, W Zerr, I Skworc, K Arendt, S Oberdieck, C Bodemer, M Poser, S Kretzschmar, HA Univ Gottingen Inst Neuropathol D-37075 Gottingen Germany Neurol Klin Gottingen Germany

Human prion diseases may be acquired as infectious diseases, they may be inherited in an autosomal dominant fashion or occur sporadically. Mutations and polymorphisms in the sequence of the coding region of the prion protein gene (PRNP) have been established as an important factor in all of these three types of prion diseases. Therefore, a total of 578 patients with suspect prion diseases referred to the German Creutzfeldt-Jakob disease (CJD) surveillance unit over a period of 4.5 years have been examined for mutations and polymorphisms in the coding region of PRNP. We found 40 cases with a missense mutation previously reported as pathogenic. Amongst these, the aspartate to asparagine change at codon 178 (D178N) was the most common mutation. All of these cases carried the D178N mutation in coupling with methionine at codon 129, resulting in the typical fatal familial insomnia (FFI) genotype. Most cases with pathogenic mutations were not found in the group of clinically "probable" cases according to established clinical criteria, supporting the notion that inherited prion diseases often exhibit atypical features. Two novel missense mutations (T188R and P238S) and several silent polymorphisms were found, demonstrating the quality of our screening procedure based on a modified version of the single-stranded conformational polymorphism technique. In "definite" CJD cases with no pathogenic mutation, the patients clinically classified as "probable" were mostly homozygous for methionine at the common polymorphism at codon 129, whereas there was a marked over-representation of patients homozygous for valine amongst those clinically classified as "possible". This large study on suspect cases of human prion diseases in Germany clearly shows that PRNP genetics is essential for a comprehensive analysis of prion diseases.

关键词： DNA/化学 DNA/遗传学 DNA突变分析基因型德国/流行病学发病率诱变插入突变突变误义多态现象遗传朊病毒病/流行病学朊病毒病/遗传学朊病毒/遗传学人类

来源：评论

学校读者我要写书评

暂无评论

Ecosystems supporting clusters of sporadic TSEs demonstrate excesses of the radical-generating divalent cation manganese and deficiencies of antioxidant co factors Co, Se, Fe, Zn - Does a foreign cation substitution at prion protein's Cu domain initiate TSE?

引用

MEDICAL HYPOTHESES 2000年第2期54卷 278-306页

作者： Purdey, M High Barn Farm Taunton TA4 3PX Somerset England

Analyses of food chains supporting isolated clusters of sporadic TSEs (CWD in N Colorado, scrapie in Iceland, CJD in Slovakia) demonstrate a consistent 2 1/2+ fold greater concentration of the pro-oxidant divalent cation, manganese (Mn), in relation to normal levels recorded in adjoining TSE-free localities. Deficiencies of the antioxidant co factors Cu/Se/Zn/Fe and Mg, P and Na were also consistently recorded in TSE foodchains. Similarities between the clinical/pathological profile of TSEs and Mn delayed psycho-neurotoxicity in miners are cited, and a novel theory generated which suggests that sporadic TSE results from early life dependence of TSE susceptible genotypes on ecosytems characterised by this specific pattern of mineral imbalance. Low Cu/Fe induces an excessive absorption of Mn in ruminants and an increased oxidation of Mn2+ into its pro oxidant species, Mn3+, which accumulates in mitochondria of CNS astrocytes in Mn SOD deficient genotypes. Deficiencies of scavenger co factors Cu/Zn/Se/Fe in the CNS permits Mn3+ initiated chain reactions of auto-oxidant mediated neuronal degeneration to proliferate, which, in turn, up-regulates the expression of the Cu-metalloprotein, prion protein (PrP). Once the rate of PrP turnover and its demand for Cu exceeds the already depleted supply of Cu within the CNS, PrP can no longer bind sufficient Cu to maintain its conformation. Mn3+ substitutes at the vacated Gu domain on PrP, thus priming up a latent capacity for lethal auto-oxidative activity to be carried along with PrP like a 'trojan horse';where Mn3+ serves as the integral 'infectious' transmissible component of the misfolded PrP-cation complex. The Mn overactivation of concanavalin A binding to glycoprotein and Mn-initiated autoxidation results in a diverse pathological profile involving receptor capping, aggregation/modification of CNS membrane/cytoskeletal proteins. TSE ensues. The BSE/nv CJD strain entails a 'synthetic' induction of the same CNS mineral distur

关键词：氨基酸序列抗氧化剂/代谢阳离子二价铜/缺乏克-亚综合征/流行病学克-亚综合征/病因学疾病暴发流行生态系统脑病牛海绵状/流行病学脑病牛海绵状/病因学自由基铁/缺乏模型生物学分子序列数据朊病毒病/流行病学朊病毒病/病因学朊病毒/化学羊瘙痒症/流行病学羊瘙痒症/病因学硒/缺乏绵羊锌/缺乏动物牛人类

来源：评论

学校读者我要写书评

暂无评论

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

请选择保存的检索档案：

请选择收藏分类：

通借通还

建议与咨询 留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

请选择保存的检索档案： 新增检索档案 确定 取消

请选择收藏分类： 新增自定义分类 确定 取消

通借通还

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

请选择保存的检索档案：

请选择收藏分类：