IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1859439

V A Siniukova, J V Sopova, S A Galkina, A P Galkin

引用次数: 9

A patient with spastic paralysis finally diagnosed as V180I genetic Creutzfeldt-Jakob disease 9 years after onset. 一例痉挛性麻痹患者在发病9年后最终被诊断为V180I遗传性克雅氏病。

IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1823179

Taichi Nomura, Ikuko Iwata, Ryoji Naganuma, Masaaki Matsushima, Katsuya Satoh, Tetsuyuki Kitamoto, Ichiro Yabe

{"title":"A patient with spastic paralysis finally diagnosed as V180I genetic Creutzfeldt-Jakob disease 9 years after onset.","authors":"Taichi Nomura, Ikuko Iwata, Ryoji Naganuma, Masaaki Matsushima, Katsuya Satoh, Tetsuyuki Kitamoto, Ichiro Yabe","doi":"10.1080/19336896.2020.1823179","DOIUrl":"10.1080/19336896.2020.1823179","url":null,"abstract":"ABSTRACT Genetic Creutzfeldt-Jakob disease (gCJD) with a mutation in codon 180 of the prion protein gene (V180I gCJD) is the most common form of gCJD in Japan, but only a few cases have been reported in Europe and the United States. It is clinically characterized by occurring in the elderly and presenting as slowly progressive dementia, although it generally shows less cerebellar and pyramidal symptoms than sporadic CJD. Here, we report a patient with V180I gCJD who initially presented with slowly progressive spastic paralysis with neither cerebrospinal fluid (CSF) nor magnetic resonance imaging (MRI) abnormalities. His symptoms progressed gradually, and after 9 years, he displayed features more typical of CJD. Diffusion-weighted MRI revealed high-intensity signals in the cortical gyrus, and there was a marked increase of 14-3-3 protein and total tau protein in the CSF, but he was negative for the real-time quaking-induced conversion assay. Although the time course was more consistent with Gerstmann-Sträussler-Scheinker disease than CJD, genetic testing revealed V180I gCJD. This is the first report of a patient with V180I gCJD who initially presented with spastic paralysis, and also the first to reveal that it took 9 years from disease onset for cortical dysfunction to develop and for MRI and CSF abnormalities to be detectable. In conclusion, we should screen for V180I gCJD in elderly patients presenting with slowly progressive spastic paralysis.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2020.1823179","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38386640","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: 3

Proteinase K resistant cores of prions and amyloids. 朊病毒和淀粉样蛋白的蛋白酶K抗性核心。

IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2019.1704612

Vitaly V Kushnirov, Alexander A Dergalev, Alexander I Alexandrov

引用次数: 29

RT-QuIC detection of tauopathies using full-length tau substrates. 使用全长 tau 底物 RT-QuIC 检测 tauopathies。

IF 1.9 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1832946

Joanne M Tennant, Davin M Henderson, Thomas M Wisniewski, Edward A Hoover

引用次数: 0

Determination of amyloid core regions of insulin analogues fibrils. 胰岛素类似物原纤维淀粉样蛋白核心区域的测定。

IF 2.3 3区生物学

Prion Pub Date : 2020-01-01 DOI: 10.1080/19336896.2020.1776062

Alexey K Surin, Sergei Yu Grishin, Oxana V Galzitskaya

引用次数: 10

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

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":null,"pages":null},"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

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":null,"pages":null},"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

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

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

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