Prion最新文献_第9页

Microbial specialization by prions. 朊病毒的微生物特化

IF 2.3 3区生物学

Prion Pub Date : 2018-07-24 DOI: 10.1080/19336896.2018.1469945

Gregory A Newby, Can Kayatekin

引用次数: 0

Effects of peptidyl-prolyl isomerase 1 depletion in animal models of prion diseases. 肽基脯氨酸异构酶1耗竭对朊病毒疾病动物模型的影响。

IF 2.3 3区生物学

Prion Pub Date : 2018-03-04 Epub Date: 2018-05-18 DOI: 10.1080/19336896.2018.1464367

Giuseppe Legname, Tommaso Virgilio, Edoardo Bistaffa, Chiara Maria Giulia De Luca, Marcella Catania, Paola Zago, Elisa Isopi, Ilaria Campagnani, Fabrizio Tagliavini, Giorgio Giaccone, Fabio Moda

引用次数: 4

THERPA: A small molecule database related to prion protein regulation and prion diseases progression. THERPA：与朊病毒蛋白调控和朊病毒疾病进展相关的小分子数据库。

IF 2.3 3区生物学

Prion Pub Date : 2018-03-04 Epub Date: 2018-05-04 DOI: 10.1080/19336896.2018.1461519

Sol Moe Lee, Wonseok Lee, Yeong Seon Lee, Jin-Soo Yoo, Soo-Jung Park, Heebal Kim, Su Yeon Kim

引用次数: 0

Design, implementation, and interpretation of amplification studies for prion detection. 设计、实施和解释朊病毒检测的扩增研究。

IF 2.3 3区生物学

Prion Pub Date : 2018-03-04 Epub Date: 2018-03-09 DOI: 10.1080/19336896.2018.1443000

Nicholas J Haley, Jürgen A Richt, Kristen A Davenport, Davin M Henderson, Edward A Hoover, Matteo Manca, Byron Caughey, Douglas Marthaler, Jason Bartz, Sabine Gilch

{"title":"Design, implementation, and interpretation of amplification studies for prion detection.","authors":"Nicholas J Haley, Jürgen A Richt, Kristen A Davenport, Davin M Henderson, Edward A Hoover, Matteo Manca, Byron Caughey, Douglas Marthaler, Jason Bartz, Sabine Gilch","doi":"10.1080/19336896.2018.1443000","DOIUrl":"https://doi.org/10.1080/19336896.2018.1443000","url":null,"abstract":"Amplification assays for transmissible spongiform encephalopathies have been in development for close to 15 years, with critical implications for the postmortem and antemortem diagnosis of human and animal prion diseases. Little has been published regarding the structured development, implementation and interpretation of experiments making use of protein misfolding cyclic amplification (PMCA) and real time quaking-induced conversion (RT-QuIC), and our goal with this Perspectives manuscript is to offer a framework which might allow for more efficient expansion of pilot studies into diagnostic trials in both human and animal subjects. This framework is made up of approaches common to diagnostic medicine, including a thorough understanding of analytical and diagnostic sensitivity and specificity, an a priori development of amplification strategy, and an effective experimental design. It is our hope that a structured framework for prion amplification assays will benefit not only experiments seeking to sensitively detect naturally-occurring cases of prion diseases and describe the pathogenesis of TSEs, but ultimately assist with future endeavors seeking to use these methods more broadly for other protein misfolding disorders, including Alzheimer's and Parkinson's disease.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"12 2","pages":"73-82"},"PeriodicalIF":2.3,"publicationDate":"2018-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2018.1443000","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35853485","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: 8

Behind the potential evolution towards prion resistant species. 在向朊病毒抗性物种进化的潜在背后。

IF 2.3 3区生物学

Prion Pub Date : 2018-03-04 Epub Date: 2018-02-20 DOI: 10.1080/19336896.2018.1435935

Natalia Fernández-Borges, Hasier Eraña, Joaquín Castilla

{"title":"Behind the potential evolution towards prion resistant species.","authors":"Natalia Fernández-Borges, Hasier Eraña, Joaquín Castilla","doi":"10.1080/19336896.2018.1435935","DOIUrl":"https://doi.org/10.1080/19336896.2018.1435935","url":null,"abstract":"Historically, the observation of naturally occurring cases of prion disease led to the classification of different susceptibility grades and to the designation of prion resistant species. However, the development of highly efficient in vitro prion propagation systems and the generation of ad hoc transgenic models allowed determining that leporidae and equidae families have been erroneously considered resistant to prion infection. On the contrary, similar approaches revealed an unexpected high level of resistance of the canidae family. In PLoS Pathogens [ 1 ], we describe experiments directed toward elucidating which are the determinants of the alleged prion resistance of this family. Studies based on the sequence of the canine prion protein coupled with structural in silico analysis identified a key residue probably implicated in this resistance. Cell and brain-based PMCA highlighted that the presence of aspartic or glutamic acid at codon 163 of the canid PrP, strongly inhibits prion replication in vitro. Transgenic animals carrying this substitution in mouse PrP were resistant to prion infection after intracerebral challenge with different mouse prion strains. The confirmation of the importance of this substitution and its exclusivity in this family, suggests it could have been evolutionarily favored, due to their diet based on carrion and small ruminants.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"12 2","pages":"83-87"},"PeriodicalIF":2.3,"publicationDate":"2018-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2018.1435935","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35782691","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: 7

Impaired transport of intrinsically disordered proteins through the Sec61 and SecY translocon; implications for prion diseases. 内在无序蛋白通过Sec61和SecY易位的转运受损;对朊病毒疾病的影响。

IF 2.3 3区生物学

Prion Pub Date : 2018-03-04 Epub Date: 2018-03-29 DOI: 10.1080/19336896.2018.1435936

Sebastian Jung, Jörg Tatzelt

{"title":"Impaired transport of intrinsically disordered proteins through the Sec61 and SecY translocon; implications for prion diseases.","authors":"Sebastian Jung, Jörg Tatzelt","doi":"10.1080/19336896.2018.1435936","DOIUrl":"https://doi.org/10.1080/19336896.2018.1435936","url":null,"abstract":"The prion protein (PrP) is composed of two major domains of similar size. The structured C-terminal domain contains three alpha-helical regions and a short two-stranded beta-sheet, while the N-terminal domain is intrinsically disordered. The analysis of PrP mutants with deletions in the C-terminal globular domain provided the first hint that intrinsically disordered domains are inefficiently transported into the endoplasmic reticulum through the Sec61 translocon. Interestingly, C-terminally truncated PrP mutants have been linked to inherited prion disease in humans and are characterized by inefficient ER import and the formation of neurotoxic PrP conformers. In a recent study we found that the Sec61 translocon in eukaryotic cells as well as the SecY translocon in bacteria is inherently deficient in translocating intrinsically disordered proteins. Moreover, our results suggest that translocon-associated components in eukaryotic cells enable the Sec61 complex to transport secretory proteins with extended unstructured domains such as PrP and shadoo.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"12 2","pages":"88-92"},"PeriodicalIF":2.3,"publicationDate":"2018-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2018.1435936","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35783980","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

Prolonged follicular helper T cell responses in ME7 scrapie-infected mice. ME7瘙痒病感染小鼠滤泡辅助性T细胞反应延长。

IF 2.3 3区生物学

Prion Pub Date : 2018-03-04 Epub Date: 2018-04-20 DOI: 10.1080/19336896.2018.1458573

Soochan Kim, Sinsuk Han, Taehyun Kim, Jeehoon Nam, Yong-Sun Kim, Eun-Kyoung Choi, Mi-Yeon Kim

{"title":"Prolonged follicular helper T cell responses in ME7 scrapie-infected mice.","authors":"Soochan Kim, Sinsuk Han, Taehyun Kim, Jeehoon Nam, Yong-Sun Kim, Eun-Kyoung Choi, Mi-Yeon Kim","doi":"10.1080/19336896.2018.1458573","DOIUrl":"https://doi.org/10.1080/19336896.2018.1458573","url":null,"abstract":"We previously reported that mice intracerebrally inoculated with the mouse-adapted scrapie strain ME7 have markedly diminished T zones in the spleen due to the decreased expression of CCL19 and CCL21. In addition, follicular dendritic cell networks in germinal centers were larger in ME7-infected spleens compared to uninfected spleens. As an extension of that study, we set out to determine how ME7 infection affects spleen structure and follicular helper T (Tfh) cell responses in mice. For this study, mice were intraperitoneally inoculated with brain homogenate of the ME7 inoculum and spleens were analyzed 50, 130, and 200 days after inoculation and compared with those from uninfected mice. The result showed that ME7- infected mice had increased Tfh cell responses which were maintained until end-stage prion disease. Although CD4 T cells decreased in white pulps, they increased in germinal centers, and expressed higher levels of the Tfh-related genes, such as Bcl6, Il21, Cxcr5, Icos, and Pdcd1. In addition, ME7-infected spleens had increased numbers of CD4 memory T cells. These data indicate that although ME7 infection led to impaired splenic white pulp structure, CD4 memory T cells were increased and Tfh cell responses were required and prolonged to provide help for the replication and accumulation of pathogenic prion protein in germinal centers.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"12 2","pages":"109-116"},"PeriodicalIF":2.3,"publicationDate":"2018-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2018.1458573","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35976370","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

Role of Prion protein-EGFR multimolecular complex during neuronal differentiation of human dental pulp-derived stem cells. 朊蛋白- egfr多分子复合物在人牙髓源性干细胞神经元分化中的作用。

IF 2.3 3区生物学

Prion Pub Date : 2018-03-04 Epub Date: 2018-05-04 DOI: 10.1080/19336896.2018.1463797

Stefano Martellucci, Valeria Manganelli, Costantino Santacroce, Francesca Santilli, Luca Piccoli, Maurizio Sorice, Vincenzo Mattei

{"title":"Role of Prion protein-EGFR multimolecular complex during neuronal differentiation of human dental pulp-derived stem cells.","authors":"Stefano Martellucci, Valeria Manganelli, Costantino Santacroce, Francesca Santilli, Luca Piccoli, Maurizio Sorice, Vincenzo Mattei","doi":"10.1080/19336896.2018.1463797","DOIUrl":"https://doi.org/10.1080/19336896.2018.1463797","url":null,"abstract":"Cellular prion protein (PrPC) is expressed in a wide variety of stem cells in which regulates their self-renewal as well as differentiation potential. In this study we investigated the presence of PrPC in human dental pulp-derived stem cells (hDPSCs) and its role in neuronal differentiation process. We show that hDPSCs expresses early PrPC at low concentration and its expression increases after two weeks of treatment with EGF/bFGF. Then, we analyzed the association of PrPC with gangliosides and EGF receptor (EGF-R) during neuronal differentiation process. PrPC associates constitutively with GM2 in control hDPSCs and with GD3 only after neuronal differentiation. Otherwise, EGF-R associates weakly in control hDPSCs and more markedly after neuronal differentiation. To analyze the functional role of PrPC in the signal pathway mediated by EGF/EGF-R, a siRNA PrP was applied to ablate PrPC and its function. The treatment with siRNA PrP significantly prevented Akt and ERK1/2 phosphorylation induced by EGF. Moreover, siRNA PrP treatment significantly prevented neuronal-specific antigens expression induced by EGF/bFGF, indicating that cellular prion protein is essential for EGF/bFGF-induced hDPSCs differentiation. These results suggest that PrPC interact with EGF-R within lipid rafts, playing a role in the multimolecular signaling complexes involved in hDPSCs neuronal differentiation.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"12 2","pages":"117-126"},"PeriodicalIF":2.3,"publicationDate":"2018-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2018.1463797","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36000090","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: 23

Chronic wasting disease management in ranched elk using rectal biopsy testing. 使用直肠活检检测牧场麋鹿慢性消耗性疾病的管理。

IF 2.3 3区生物学

Prion Pub Date : 2018-03-04 Epub Date: 2018-02-22 DOI: 10.1080/19336896.2018.1436925

Nicholas J Haley, Davin M Henderson, Sarah Wycoff, Joanne Tennant, Edward A Hoover, Dan Love, Ed Kline, Aaron Lehmkuhl, Bruce Thomsen

{"title":"Chronic wasting disease management in ranched elk using rectal biopsy testing.","authors":"Nicholas J Haley, Davin M Henderson, Sarah Wycoff, Joanne Tennant, Edward A Hoover, Dan Love, Ed Kline, Aaron Lehmkuhl, Bruce Thomsen","doi":"10.1080/19336896.2018.1436925","DOIUrl":"https://doi.org/10.1080/19336896.2018.1436925","url":null,"abstract":"Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) affecting members of the cervid species, and is one of the few TSEs with an expanding geographic range. Diagnostic limitations, efficient transmission, and the movement of infected animals are important contributing factors in the ongoing spread of disease. Managing CWD in affected populations has proven difficult, relying on population reduction in the case of wild deer and elk, or quarantine and depopulation in farmed cervids. In the present study, we evaluated the effectiveness of managing endemic CWD in a closed elk herd using antemortem sampling combined with both conventional and experimental diagnostic testing, and selective, targeted culling of infected animals. We hypothesized that the real-time quaking-induced conversion (RT-QuIC) assay, a developing amplification assay, would offer greater detection capabilities over immunohistochemistry (IHC) in the identification of infected animals using recto-anal mucosa associated lymphoid tissue (RAMALT). We further sought to develop a better understanding of CWD epidemiology in elk with various PRNP alleles, and predicted that CWD prevalence would decrease with targeted culling. We found that RT-QuIC identified significantly more CWD-positive animals than IHC using RAMALT tissues (121 vs. 86, respectively, out of 553 unique animals), and that longstanding disease presence was associated with an increasing frequency of less susceptible PRNP alleles. Prevalence of CWD increased significantly over the first two years of the study, implying that refinements in our management strategy are necessary to reduce the prevalence of CWD in this herd.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"12 2","pages":"93-108"},"PeriodicalIF":2.3,"publicationDate":"2018-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2018.1436925","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35814828","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: 31

Wernicke-Korsakoff syndrome as a rare phenotype of sporadic Creutzfeldt-Jakob disease. wernickke - korsakoff综合征是散发性克雅氏病的一种罕见表型。

IF 2.3 3区生物学

Prion Pub Date : 2018-03-04 Epub Date: 2018-02-09 DOI: 10.1080/19336896.2018.1433988

Joanna Bielewicz, Anna Szczepańska-Szerej, Magdalena Ogórek, Piotr Dropko, Katarzyna Wojtal, Konrad Rejdak

引用次数: 1