Prion最新文献_第10页

PRION 2019 emerging concepts 朊病毒2019新兴概念

IF 2.3 3区生物学

Prion Pub Date : 2019-05-15 DOI: 10.1080/19336896.2019.1615197

Meyer-Luehmann, C. Sigurdson, M. Jucker

引用次数: 2

Deciphering the BSE-type specific cell and tissue tropisms of atypical (H and L) and classical BSE 解读非典型（H和L）和经典BSE的BSE类型特异性细胞和组织倾向

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1651180

A. Balkema-Buschmann, Grit Priemer, R. Ulrich, R. Strobelt, Bob Hills, M. Groschup

{"title":"Deciphering the BSE-type specific cell and tissue tropisms of atypical (H and L) and classical BSE","authors":"A. Balkema-Buschmann, Grit Priemer, R. Ulrich, R. Strobelt, Bob Hills, M. Groschup","doi":"10.1080/19336896.2019.1651180","DOIUrl":"https://doi.org/10.1080/19336896.2019.1651180","url":null,"abstract":"ABSTRACT After the discovery of two atypical bovine spongiform encephalopathy (BSE) forms in France and Italy designated H- and L-BSE, the question arose whether these new forms differed from classical BSE (C-BSE) in their pathogenesis. Samples collected from cattle in the clinical stage of BSE during an intracranial challenge study with L- and H-BSE were analysed using biochemical and histological methods as well as in a transgenic mouse bioassay. Our results generally confirmed what had been described for C-BSE to be true also for both atypical BSE forms, namely the restriction of the pathological prion protein (PrPSc) and BSE infectivity to the nervous system. However, analysis of samples collected under identical conditions from both atypical H- and L-BSE forms allowed us a more precise assessment of the grade of involvement of different tissues during the clinical end stage of disease as compared to C-BSE. One important feature is the involvement of the peripheral nervous and musculoskeletal tissues in both L-BSE and H-BSE affected cattle. We were, however, able to show that in H-BSE cases, the PrPSc depositions in the central and peripheral nervous system are dominated by a glial pattern, whereas a neuronal deposition pattern dominates in L-BSE cases, indicating differences in the cellular and topical tropism of both atypical BSE forms. As a consequence of this cell tropism, H-BSE seems to spread more rapidly from the CNS into the periphery via the glial cell system such as Schwann cells, as opposed to L-BSE which is mostly propagated via neuronal cells.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"13 1","pages":"160 - 172"},"PeriodicalIF":2.3,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2019.1651180","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43458636","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 8

Prion-dependent proteome remodeling in response to environmental stress is modulated by prion variant and genetic background. 朊病毒依赖性蛋白质组重塑对环境胁迫的响应是由朊病毒变异和遗传背景调节的。

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1583041

Ben Allwein, Christina Kelly, Shaima Kammoonah, Thibault Mayor, Dale M Cameron

{"title":"Prion-dependent proteome remodeling in response to environmental stress is modulated by prion variant and genetic background.","authors":"Ben Allwein, Christina Kelly, Shaima Kammoonah, Thibault Mayor, Dale M Cameron","doi":"10.1080/19336896.2019.1583041","DOIUrl":"https://doi.org/10.1080/19336896.2019.1583041","url":null,"abstract":"A number of fungal proteins are capable of adopting multiple alternative, self-perpetuating prion conformations. These prion variants are associated with functional alterations of the prion-forming protein and thus the generation of new, heritable traits that can be detrimental or beneficial. Here we sought to determine the extent to which the previously-reported ZnCl2-sensitivity trait of yeast harboring the [PSI+] prion is modulated by genetic background and prion variant, and whether this trait is accompanied by prion-dependent proteomic changes that could illuminate its physiological basis. We also examined the degree to which prion variant and genetic background influence other prion-dependent phenotypes. We found that ZnCl2 exposure not only reduces colony growth but also limits chronological lifespan of [PSI+] relative to [psi-] cells. This reduction in viability was observed for multiple prion variants in both the S288C and W303 genetic backgrounds. Quantitative proteomic analysis revealed that under exposure to ZnCl2 the expression of stress response proteins was elevated and the expression of proteins involved in energy metabolism was reduced in [PSI+] relative to [psi-] cells. These results suggest that cellular stress and slowed growth underlie the phenotypes we observed. More broadly, we found that prion variant and genetic background modulate prion-dependent changes in protein abundance and can profoundly impact viability in diverse environments. Thus, access to a constellation of prion variants combined with the accumulation of genetic variation together have the potential to substantially increase phenotypic diversity within a yeast population, and therefore to enhance its adaptation potential in changing environmental conditions.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"13 1","pages":"53-64"},"PeriodicalIF":2.3,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2019.1583041","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36565738","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 2

In vitro generation of tau aggregates conformationally distinct from parent tau seeds of Alzheimer's brain. 体外生成的tau聚集体构象不同于阿尔茨海默病大脑的母体tau种子。

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 Epub Date: 2018-11-14 DOI: 10.1080/19336896.2018.1545524

Won-Hee Nam, Young Pyo Choi

引用次数: 9

Use of faecal volatile organic compound analysis for ante-mortem discrimination between CWD-positive, -negative exposed, and -known negative white-tailed deer (Odocoileus virginianus). 利用粪便挥发性有机化合物分析法在死前对白尾鹿（Odocoileus virginianus）的化脓性白尾鹿病（CWD）阳性、暴露阴性和已知阴性进行鉴别。

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1607462

Christine K Ellis, Steven F Volker, Doreen L Griffin, Kurt C VerCauteren, Tracy A Nichols

引用次数: 0

The worst is yet to come: probable sporadic Creutzfeldt-Jakob disease in a well-controlled HIV patient. 最糟糕的情况还在后头:一名控制良好的艾滋病患者可能会出现散发性克雅氏病

IF 1.9 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1648985

Euripedes Gomes De Carvalho Neto, Matheus Ferreira Gomes, Marina De Oliveira, Maryuris Isabel Niño Guete, Iuri Pereira Santos, Mateus Damiani Monteiro, Fernando Gustavo Stelzer, Fernando Kowacs, Liselotte Menke Barea

引用次数: 0

A case of V180I genetic Creutzfeldt-Jakob disease presenting with conspicuous facial mimicry 一例V180I基因型克雅氏病表现为明显的面部模仿

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1651181

Y. Iwasaki, K. Mori, Masumi Ito, Y. Kawai

{"title":"A case of V180I genetic Creutzfeldt-Jakob disease presenting with conspicuous facial mimicry","authors":"Y. Iwasaki, K. Mori, Masumi Ito, Y. Kawai","doi":"10.1080/19336896.2019.1651181","DOIUrl":"https://doi.org/10.1080/19336896.2019.1651181","url":null,"abstract":"ABSTRACT Although there have been no reports of facial mimicry in patients with Creutzfeldt-Jakob disease (CJD), we encountered a patient with genetic CJD with prion protein gene codon 180 mutation (V180I gCJD) who apparently showed this interesting clinical finding. The patient was an 87-year-old Japanese woman, and the first observed CJD symptom was poor spontaneity. She gradually showed cognitive dysfunction and subsequently gait disturbance. A prion protein gene analysis revealed a V180I mutation with methionine homozygosity at codon 129. Facial mimicry was observed 7 months after disease onset and continued for approximately 9 months. Pathological laughing and startle reaction were also observed during approximately the same period, whereas myoclonus was observed at a later stage, 12 months after disease onset, and was very mild in degree. Electroencephalography studies showed a diffuse slow basic pattern without periodic sharp wave complexes. Diffusion-weighted magnetic resonance imaging showed extensive hyperintensity in the cerebral cortex, and there was also hyperintensity with edematous swelling in the same regions on T2-weighted and fluid-attenuated inversion recovery images. On the basis of the magnetic resonance imaging findings and the findings of previous case reports of V180I gCJD, we speculate that the characteristic extensive cerebrocortical involvement observed in V180I gCJD was implicated in the pathogenesis of the facial mimicry observed in this case.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"13 1","pages":"151 - 155"},"PeriodicalIF":2.3,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2019.1651181","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44653426","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 6

Clinical features and genetic characteristics of two Chinese pedigrees with fatal family insomnia. 致命性家族性失眠症两家系的临床特征及遗传特征。

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1617027

Runcheng He, Yacen Hu, Lingyan Yao, Yun Tian, Yafang Zhou, Fang Yi, Lin Zhou, Hongwei Xu, Qiying Sun

{"title":"Clinical features and genetic characteristics of two Chinese pedigrees with fatal family insomnia.","authors":"Runcheng He, Yacen Hu, Lingyan Yao, Yun Tian, Yafang Zhou, Fang Yi, Lin Zhou, Hongwei Xu, Qiying Sun","doi":"10.1080/19336896.2019.1617027","DOIUrl":"https://doi.org/10.1080/19336896.2019.1617027","url":null,"abstract":"Background: Fatal familial insomnia (FFI) is a rare autosomal-dominant inherited prion disease characterized clinically by severe sleep disorder, motor signs, dysautonomia and abnormal behaviour. FFI is caused by a missense mutation at codon 178 of the prion protein gene (PRNP). Our study is aimed to explore typical clinical and genetic features of two Chinese pedigrees with FFI and review the related literatures. Methods: Two FFI cases with family histories were recruited in our study. The main clinical features, genetic features and possible pathophysiologic mechanisms of these two FFI cases were analysed. Results: The foremost symptoms seemed to be sleep disturbances and psychosis. Progressive sympathetic symptoms, movement disturbances and memory loss were frequently observed as well. Electroencephalography (EEG) showed a minor slowing without periodic triphasic waves. Polysomnography (PSG) showed reduction in total sleep time and disturbance of sleep-related respiratory. Brain magnetic resonance imaging (MRI) did not reveal obvious abnormality. Genetic analysis disclosed the prion protein gene mutation at codon 178 (D178N), with methionine (Met) homozygosity at the polymorphic position 129 (Met129Met). Conclusions: The major clinical features of Chinese FFI are sleep dysfunction, psychiatric symptoms and sympathetic symptoms. Our patients have similar clinical characteristics as that of the typical FFI cases.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"13 1","pages":"116-123"},"PeriodicalIF":2.3,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2019.1617027","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37266638","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 11

The insomnia phenotype in genetic Creutzfeldt-Jakob disease based on the E200K mutation. 基于E200K突变的遗传性克雅氏病失眠表型研究

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1590938

Eva Feketeova, Dominika Jarcuskova, Alzbeta Janakova, Marianna Vitkova, Jozef Dragasek, Zuzana Gdovinova

引用次数: 1

Combining autophagy stimulators and cellulose ethers for therapy against prion disease 结合自噬刺激剂和纤维素醚治疗朊病毒病

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1670928

Basant A. Abdulrahman, Waqas Tahir, K. Doh-ura, S. Gilch, H. Schatzl

{"title":"Combining autophagy stimulators and cellulose ethers for therapy against prion disease","authors":"Basant A. Abdulrahman, Waqas Tahir, K. Doh-ura, S. Gilch, H. Schatzl","doi":"10.1080/19336896.2019.1670928","DOIUrl":"https://doi.org/10.1080/19336896.2019.1670928","url":null,"abstract":"ABSTRACT Prion diseases are fatal transmissible neurodegenerative disorders that affect animals and humans. Prions are proteinaceous infectious particles consisting of a misfolded isoform of the cellular prion protein PrPC, termed PrPSc. PrPSc accumulates in infected neurons due to partial resistance to proteolytic digestion. Using compounds that interfere with the production of PrPSc or enhance its degradation cure prion infection in vitro, but most drugs failed when used to treat prion-infected rodents. In order to synergize the effect of anti-prion drugs, we combined drugs interfering with the generation of PrPSc with compounds inducing PrPSc degradation. Here, we tested autophagy stimulators (rapamycin or AR12) and cellulose ether compounds (TC-5RW or 60SH-50) either as single or combination treatment of mice infected with RML prions. Single drug treatments significantly extended the survival compared to the untreated group. As anticipated, also all the combination therapy groups showed extended survival compared to the untreated group, but no combination treatment showed superior effects to 60SH-50 or TC-5RW treatment alone. Unexpectedly, we later found that combining autophagy stimulator and cellulose ether treatment in cultured neuronal cells mitigated the pro-autophagic activity of AR12 and rapamycin, which can in part explain the in vivo results. Overall, we show that it is critical to exclude antagonizing drug effects when attempting combination therapy. In addition, we identified AR-12 as a pro-autophagic drug that significantly extends survival of prion-infected mice, has no adverse side effects on the animals used in this study, and can be useful in future studies.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"13 1","pages":"185 - 196"},"PeriodicalIF":2.3,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2019.1670928","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42979573","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 19