IF 1.9 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1714373

Lindsay E Parrie, Jenna A E Crowell, Julie A Moreno, Stephanie S Suinn, Glenn C Telling, Richard A Bessen

{"title":"The cellular prion protein promotes neuronal regeneration after acute nasotoxic injury.","authors":"Lindsay E Parrie, Jenna A E Crowell, Julie A Moreno, Stephanie S Suinn, Glenn C Telling, Richard A Bessen","doi":"10.1080/19336896.2020.1714373","DOIUrl":"10.1080/19336896.2020.1714373","url":null,"abstract":"Adult neurogenesis, analogous to early development, is comprised of several, often concomitant, processes including proliferation, differentiation, and formation of synaptic connections. However, due to continual, asynchronous turn-over, newly-born adult olfactory sensory neurons (OSNs) must integrate into existing circuitry. Additionally, OSNs express high levels of cellular prion protein (PrPC), particularly in the axon, which implies a role in this cell type. The cellular prion has been shown to be important for proper adult OSN neurogenesis primarily by stabilizing mature olfactory neurons within this circuitry. However, the role of PrPC on each specific adult neurogenic processes remains to be investigated in detail. To tease out the subtle effects of prion protein expression level, a large population of regenerating neurons must be investigated. The thyroid drug methimazole (MTZ) causes nearly complete OSN loss in rodents and is used as a model of acute olfactory injury, providing a mechanism to induce synchronized OSN regeneration. This study investigated the effect of PrPC on adult neurogenesis after acute nasotoxic injury. Altered PrPC levels affected olfactory sensory epithelial (OSE) regeneration, cell proliferation, and differentiation. Attempts to investigate the role of PrPC level on axon regeneration did not support previous studies, and glomerular targeting did not recover to vehicle-treated levels, even by 20 weeks. Together, these studies demonstrate that the cellular prion protein is critical for regeneration of neurons, whereby increased PrPC levels promote early neurogenesis, and that lack of PrPC delays the regeneration of this tissue after acute injury.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"14 1","pages":"31-41"},"PeriodicalIF":1.9,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6984647/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10764521","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: 0

Cross-validation of the RT-QuIC assay for the antemortem detection of chronic wasting disease in elk. RT-QuIC法在麋鹿慢性消耗性疾病死前检测中的交叉验证

IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1716657

N J Haley, R Donner, D M Henderson, J Tennant, E A Hoover, M Manca, B Caughey, N Kondru, S Manne, A Kanthasamay, S Hannaoui, S C Chang, S Gilch, S Smiley, G Mitchell, A D Lehmkuhl, B V Thomsen

{"title":"Cross-validation of the RT-QuIC assay for the antemortem detection of chronic wasting disease in elk.","authors":"N J Haley, R Donner, D M Henderson, J Tennant, E A Hoover, M Manca, B Caughey, N Kondru, S Manne, A Kanthasamay, S Hannaoui, S C Chang, S Gilch, S Smiley, G Mitchell, A D Lehmkuhl, B V Thomsen","doi":"10.1080/19336896.2020.1716657","DOIUrl":"https://doi.org/10.1080/19336896.2020.1716657","url":null,"abstract":"Chronic wasting disease is a progressively fatal, horizontally transmissible prion disease affecting several members of the cervid species. Conventional diagnosis relies on ELISA or IHC evaluation using tissues collected post-mortem; however, recent research has focused on newly developed amplification techniques using samples collected antemortem. The present study sought to cross-validate the real-time quaking-induced conversion assay (RT-QuIC) evaluation of rectal biopsies collected from an elk herd with endemic CWD, assessing both binary positive/negative test results as well as relative rates of amplification between laboratories. We found that results were correlative in both categories across all laboratories performing RT-QuIC, as well as to conventional IHC performed at a national reference laboratory. A significantly higher number of positive samples were identified using RT-QuIC, with results seemingly unhindered by low follicle counts. These findings support the continued development and implementation of amplification assays in the diagnosis of prion diseases of veterinary importance, targeting not just antemortem sampling strategies, but post-mortem testing approaches as well.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"14 1","pages":"47-55"},"PeriodicalIF":2.3,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2020.1716657","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10764520","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: 19

Management of chronic wasting disease in ranched elk: conclusions from a longitudinal three-year study. 牧场麋鹿慢性消耗性疾病的管理:一项为期三年的纵向研究的结论。

IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1724754

N J Haley, D M Henderson, R Donner, S Wyckoff, K Merrett, J Tennant, E A Hoover, D Love, E Kline, A D Lehmkuhl, B V Thomsen

{"title":"Management of chronic wasting disease in ranched elk: conclusions from a longitudinal three-year study.","authors":"N J Haley, D M Henderson, R Donner, S Wyckoff, K Merrett, J Tennant, E A Hoover, D Love, E Kline, A D Lehmkuhl, B V Thomsen","doi":"10.1080/19336896.2020.1724754","DOIUrl":"https://doi.org/10.1080/19336896.2020.1724754","url":null,"abstract":"Chronic wasting disease is a fatal, horizontally transmissible prion disease of cervid species that has been reported in free-ranging and farmed animals in North America, Scandinavia, and Korea. Like other prion diseases, CWD susceptibility is partly dependent on the sequence of the prion protein encoded by the host's PRNP gene; it is unknown if variations in PRNP have any meaningful effects on other aspects of health. Conventional diagnosis of CWD relies on ELISA or IHC testing of samples collected post-mortem, with recent efforts focused on antemortem testing approaches. We report on the conclusions of a study evaluating the role of antemortem testing of rectal biopsies collected from over 570 elk in a privately managed herd, and the results of both an amplification assay (RT-QuIC) and conventional IHC among animals with a several PRNP genotypes. Links between PRNP genotype and potential markers of evolutionary fitness, including pregnancy rates, body condition, and annual return rates were also examined. We found that the RT-QuIC assay identified significantly more CWD positive animals than conventional IHC across the course of the study, and was less affected by factors known to influence IHC sensitivity - including follicle count and PRNP genotype. We also found that several evolutionary markers of fitness were not adversely correlated with specific PRNP genotypes. While the financial burden of the disease in this herd was ultimately unsustainable for the herd owners, our scientific findings and the hurdles encountered will assist future CWD management strategies in both wild and farmed elk and deer.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"14 1","pages":"76-87"},"PeriodicalIF":2.3,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2020.1724754","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10764522","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: 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

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

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