IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1569451

Hasier Eraña

{"title":"The Prion 2018 round tables (II): Aβ, tau, α-synuclein… are they prions, prion-like proteins, or what?","authors":"Hasier Eraña","doi":"10.1080/19336896.2019.1569451","DOIUrl":"https://doi.org/10.1080/19336896.2019.1569451","url":null,"abstract":"The description of prions as causal agents of Transmissible Spongiform Encephalopathies (TSE), is nowadays accepted as an important breakthrough in biology as revealed the existence of a completely new group of pathogens and a new way of transmission for biological information. A common feature of many neurodegenerative disorders is the presence of protein aggregates in the nervous system and as evidences highlighting the similarities of these proteins with TSE-causing prions increase, the line separating the infectious prions from other protein aggregates becomes thinner than previously thought. However, instead of encompassing all these amyloidogenic proteins under the umbrella term \"prion\", new terminology has raised including the terms prion-like, prionoid, quasi-prion or propagon. The International Prion Conference held in Santiago de Compostela in 2018, offered the perfect forum to discuss this topic and maybe set the basis for an agreed terminology. For that, a round table was organized with several experts on the field to discuss whether AÎ², tau, Î±-synuclein and others are prions, prion-like proteins, or should be named otherwise. This commentary intends to summarize the topics discussed at the round table and shed some light on this controverted topic, drawing together the opinions of many experts participating at the session.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"13 1","pages":"41-45"},"PeriodicalIF":2.3,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2019.1569451","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36910586","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: 17

Enrichment of miR-126 enhances the effects of endothelial progenitor cell-derived microvesicles on modulating MC3T3-E1 cell function via Erk1/2-Bcl-2 signalling pathway. miR-126的富集增强了内皮祖细胞来源的微泡通过Erk1/2-Bcl-2信号通路调节MC3T3-E1细胞功能的作用。

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1607464

Guanghua Chen, Peng Li, Zhijun Liu, Rong Zeng, Xiaotang Ma, Yanfang Chen, Haijia Xu, Zhanghua Li, Hao Lin

{"title":"Enrichment of miR-126 enhances the effects of endothelial progenitor cell-derived microvesicles on modulating MC3T3-E1 cell function via Erk1/2-Bcl-2 signalling pathway.","authors":"Guanghua Chen, Peng Li, Zhijun Liu, Rong Zeng, Xiaotang Ma, Yanfang Chen, Haijia Xu, Zhanghua Li, Hao Lin","doi":"10.1080/19336896.2019.1607464","DOIUrl":"https://doi.org/10.1080/19336896.2019.1607464","url":null,"abstract":"Objective: To evaluate whether EPC-MVs could promote bone regeneration by directly regulating osteoblast through miR-126. The underlying mechanisms were also explored.Methods: EPCs were isolated from bone marrow mononuclear cells. EPC-MVs were collected from EPCs cultured medium. The lentivirus was used to induce miR-126 over-expression in EPCs and EPC-MVs. miR-126 expression was detected by qRT-PCR. The proliferation, migration, apoptosis and differentiation abilities of osteoblast cells MC3T3-E1 were analysed in the presence or absence of EPC-MVs or miR-126 overexpressed EPC-MVs (EPC-MVs-miR126). The proteins of Erk1/2 and Bcl-2 were analysed by western blot. Erk1/2 inhibitor was used for pathway exploration.Results: EPC-MVs reduced apoptosis and promoted proliferation and migration of MC3T3-E1 cells, which could be enhanced by miR-126 enrichment (p< 0.05). Neither EPC-MVs nor EPC-MVs-miR126 had an effect on MC3T3-E1 cell osteogenic differentiation (p> 0.05). EPC-MVs-miR126 had better effects than EPC-MVs on upregulating the expressions of p-Erk1/2 and Bcl-2, which were abolished by Erk1/2 inhibitor. ERK1/2-Bcl-2 activity plays a crucial role in the regulation of EPC-MVs/EPC-MVs-miR126 on the effect of MC3T3-E1 cells.Conclusion: EPC-MVs promote proliferation and migration of MC3T3-E1 cell while reduced apoptosis via the miR-126/Erk1/2-Bcl-2 pathway. A combination of EPC-MVs and miR-126 might provide novel therapeutic targets for bone regeneration and fracture healing through regulating osteoblast.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"13 1","pages":"106-115"},"PeriodicalIF":2.3,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2019.1607464","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37210221","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: 9

Sensitivity and specificity evaluation of multiple neurodegenerative proteins for Creutzfeldt-Jakob disease diagnosis using a deep-learning approach. 使用深度学习方法评估多种神经退行性蛋白对克雅氏病诊断的敏感性和特异性

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1639482

Sol Moe Lee, Jae Wook Hyeon, Soo-Jin Kim, Heebal Kim, Ran Noh, Seonghan Kim, Yeong Seon Lee, Su Yeon Kim

{"title":"Sensitivity and specificity evaluation of multiple neurodegenerative proteins for Creutzfeldt-Jakob disease diagnosis using a deep-learning approach.","authors":"Sol Moe Lee, Jae Wook Hyeon, Soo-Jin Kim, Heebal Kim, Ran Noh, Seonghan Kim, Yeong Seon Lee, Su Yeon Kim","doi":"10.1080/19336896.2019.1639482","DOIUrl":"https://doi.org/10.1080/19336896.2019.1639482","url":null,"abstract":"The diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) can only be confirmed by abnormal protease-resistant prion protein accumulation in post-mortem brain tissue. The relationships between sCJD and cerebrospinal fluid (CSF) proteins such as 14-3-3, tau, and α-synuclein (a-syn) have been investigated for their potential value in pre-mortem diagnosis. Recently, deep-learning (DL) methods have attracted attention in neurodegenerative disease research. We established DL-aided pre-mortem diagnostic methods for CJD using multiple CSF biomarkers to improve their discriminatory sensitivity and specificity. Enzyme-linked immunosorbent assays were performed on phospho-tau (p-tau), total-tau (t-tau), a-syn, and β-amyloid (1-42), and western blot analysis was performed for 14-3-3 protein from CSF samples of 49 sCJD and 256 non-CJD Korean patients, respectively. The deep neural network structure comprised one input, five hidden, and one output layers, with 20, 40, 30, 20 and 12 hidden unit numbers per hidden layer, respectively. The best performing DL model demonstrated 90.38% accuracy, 83.33% sensitivity, and 92.5% specificity for the three-protein combination of t-tau, p-tau, and a-syn, and all other patients in a separate CSF set (n = 15) with other neuronal diseases were correctly predicted to not have CJD. Thus, DL-aided pre-mortem diagnosis may provide a suitable tool for discriminating CJD patients from non-CJD patients.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"13 1","pages":"141-150"},"PeriodicalIF":2.3,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2019.1639482","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37422715","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

Clinicopathological findings of an MM2-cortical-type sporadic Creutzfeldt-Jakob disease patient with cortical blindness during a course of glaucoma and age-related macular degeneration. 1例伴有青光眼和老年性黄斑变性过程中皮层性失明的mm2皮质型散发性克雅氏病患者的临床病理结果

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1631680

Yuichi Hayashi, Yasushi Iwasaki, Masahiro Waza, Hideaki Shibata, Akio Akagi, Akio Kimura, Takashi Inuzuka, Katsuya Satoh, Tetsuyuki Kitamoto, Mari Yoshida, Takayoshi Shimohata

{"title":"Clinicopathological findings of an MM2-cortical-type sporadic Creutzfeldt-Jakob disease patient with cortical blindness during a course of glaucoma and age-related macular degeneration.","authors":"Yuichi Hayashi, Yasushi Iwasaki, Masahiro Waza, Hideaki Shibata, Akio Akagi, Akio Kimura, Takashi Inuzuka, Katsuya Satoh, Tetsuyuki Kitamoto, Mari Yoshida, Takayoshi Shimohata","doi":"10.1080/19336896.2019.1631680","DOIUrl":"https://doi.org/10.1080/19336896.2019.1631680","url":null,"abstract":"Here, we report an autopsy-verified patient with MM2-coritical-type sporadic Creutzfeldt-Jakob disease (MM2C-type sCJD) presenting cortical blindness during a course of glaucoma and age-related macular degeneration, and focus on the difficulties involved in early clinical diagnosis. An 83-year-old man was admitted to our hospital 15 months after the onset of cortical blindness, and 9 months after the onset of progressive dementia. Neurological examination revealed dementia, frontal signs, visual disturbance, dysphagia, myoclonus and exaggerated tendon reflexes in the four extremities. Diffusion-weighted MRI (DW-MRI) showed cortical hyperintensities predominantly in the bilateral occipital lobes. PRNP gene analysis showed no mutations with methionine homozygosity at codon 129. Cerebrospinal fluid (CSF) examination revealed elevation of 14-3-3 and total tau protein. The symptoms progressed gradually, and the patient died of aspiration pneumonia, 30 months after the onset. Neuropathological examination revealed extensive large confluent vacuole-type spongiform changes in the cerebral cortices. Prion protein (PrP) immunostaining showed perivascular and plaque-type PrP deposits. We diagnosed our patient as MM2C-type sCJD. There are two difficulties in the early clinical diagnosis of MM2C-type sCJD with ocular disease in the elderly; delayed utilization of DW-MRI, and accompaniment of ocular disease. For early diagnosis of MM2C-type sCJD, we conclude that clinician should perform DW-MRI for patients with isolated dementia or cortical visual disturbance.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"13 1","pages":"124-131"},"PeriodicalIF":2.3,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2019.1631680","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37073074","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: 5

THERPA v2: an update of a small molecule database related to prion protein regulation and prion disease progression THERPA v2：与朊病毒蛋白调节和朊病毒疾病进展相关的小分子数据库的更新

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1689789

Sol Moe Lee, S. S. Kim, Heebal Kim, Su Yeon Kim

引用次数: 4

The prion 2018 round tables (I): the structure of PrP^Sc. 朊病毒2018圆桌会议（I）：PrPSc的结构。

IF 1.9 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1569450

Ilia V Baskakov, Byron Caughey, Jesús R Requena, Alejandro M Sevillano, Witold K Surewicz, Holger Wille

{"title":"The prion 2018 round tables (I): the structure of PrPSc.","authors":"Ilia V Baskakov, Byron Caughey, Jesús R Requena, Alejandro M Sevillano, Witold K Surewicz, Holger Wille","doi":"10.1080/19336896.2019.1569450","DOIUrl":"10.1080/19336896.2019.1569450","url":null,"abstract":"Understanding the structure of PrPSc is without doubt a sine qua non to understand not only PrPSc propagation, but also critical features of that process such as the strain phenomenon and transmission barriers. While elucidation of the PrPSc structure has been full of difficulties, we now have a large amount of structural information that allows us to begin to understand it. This commentary article summarizes a round table that took place within the Prion 2018 meeting held in Santiago de Compostela to discuss the state of the art in this matter. Two alternative models of PrPSc exist: the PIRIBS and the 4-rung β-solenoid models. Both of them have relevant features. The 4-rung β-solenoid model agrees with experimental constraints of brain derived PrPSc obtained from cryo-EM and X-ray fiber diffraction studies. Furthermore, it allows facile accommodation of the bulky glycans that decorate brain-derived PrPSc. On the other hand, the infectious PrP23-144 amyloid exhibits a PIRIBS architecture. Perhaps, both types of structure co-exist.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"13 1","pages":"46-52"},"PeriodicalIF":1.9,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6422368/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36910585","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

Sodium hydroxide treatment effectively inhibits PrP^CWD replication in farm soil. 氢氧化钠处理能有效抑制PrPCWD在农田土壤中的复制。

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1617623

Hyun-Joo Sohn, Kyung-Je Park, In-Soon Roh, Hyo-Jin Kim, Hoo-Chang Park, Hae-Eun Kang

引用次数: 5

Michael Ter-Avanesyan (1949-2018) - life in science. Michael Ter-Avanesyan(1949-2018)——科学生命。

IF 2.3 3区生物学

Prion Pub Date : 2019-01-01 DOI: 10.1080/19336896.2019.1567201

Vitaly V Kushnirov

引用次数: 1

Proteolysis: a double-edged sword for the development of amyloidoses. 蛋白水解：淀粉样变性发展的双刃剑。

IF 2.3 3区生物学

Prion Pub Date : 2018-09-09 DOI: 10.1080/19336896.2018.1521234

Atsushi Okamoto, Nao Hosoda, Shin-Ichi Hoshino

{"title":"Proteolysis: a double-edged sword for the development of amyloidoses.","authors":"Atsushi Okamoto, Nao Hosoda, Shin-Ichi Hoshino","doi":"10.1080/19336896.2018.1521234","DOIUrl":"10.1080/19336896.2018.1521234","url":null,"abstract":"The yeast Saccharomyces cerevisiae has proven to be a useful model system to investigate the mechanism of prion generation and inheritance, to which studies in Sup35 made a great contribution. Recent studies demonstrated that 'protein misfolding and aggregation' (i.e. amyloidogenesis) is a common principle underlying the pathogenesis of neurodegenerative diseases including prion, amyotrophic lateral sclerosis (ALS), Perkinson's (PD), Alzheimer's (AD) diseases and polyglutamine (polyQ) diseases such as spinocerebellar ataxia (SCA) and Hantington's disease (HD). By these findings, the yeast has again been drawing increased attention as a useful system for studying neurodegenerative proteinopathies. So far, it has been reported that proteolytic cleavage of causative amyloidogenic proteins might affect the pathogenesis of the respective neurodegenerative diseases. Although those reports provide a clear phenomenological description, in the majority of cases, it has remained elusive if proteolysis is directly involved in the pathogenesis of the diseases. Recently, we have demonstrated in yeast that proteolysis suppresses prion generation. The yeast-based strategy might make a breakthrough to the unsolved issues.","PeriodicalId":54585,"journal":{"name":"Prion","volume":" ","pages":"1-7"},"PeriodicalIF":2.3,"publicationDate":"2018-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6277189/pdf/kprn-12-5-6-1521234.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36474689","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

Unexpected prion phenotypes in experimentally transfused animals: predictive models for humans? 实验性输血动物的意外朊病毒表型：人类的预测模型？

IF 2.3 3区生物学

Prion Pub Date : 2018-08-16 DOI: 10.1080/19336896.2018.1505399

Emmanuel E Comoy, Jacqueline Mikol, Jean-Philippe Deslys

{"title":"Unexpected prion phenotypes in experimentally transfused animals: predictive models for humans?","authors":"Emmanuel E Comoy, Jacqueline Mikol, Jean-Philippe Deslys","doi":"10.1080/19336896.2018.1505399","DOIUrl":"10.1080/19336896.2018.1505399","url":null,"abstract":"The recently reevaluated high prevalence of healthy carriers (1/2,000 in UK) of variant Creutzfeldt-Jakob Disease (v-CJD), whose blood might be infectious, suggests that the evolution of this prion disease might not be under full control as expected. After experimental transfusion of macaques and conventional mice with blood derived from v-CJD exposed (human and animal) individuals, we confirmed in these both models the transmissibility of v-CJD, but we also observed unexpected neurological syndromes transmissible by transfusion: despite their prion etiology confirmed through transmission experiments, these original cases would escape classical prion diagnosis, notably in the absence of detectable abnormal PrP with current techniques. It is noteworthy that macaques developed an original, yet undescribed myelopathic syndrome associating demyelination and pseudo-necrotic lesions of spinal cord, brainstem and optical tract without affecting encephalon, which is rather evocative of spinal cord disease than prion disease in human medicine. These observations strongly suggest that the spectrum of human prion diseases may extend the current field restricted to the phenotypes associated to protease-resistant PrP, and may notably include spinal cord diseases.","PeriodicalId":54585,"journal":{"name":"Prion","volume":" ","pages":"1-8"},"PeriodicalIF":2.3,"publicationDate":"2018-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6277188/pdf/kprn-12-3-4-1505399.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36370780","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

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