Prion最新文献 - Book学术

Exploring CJD incidence trends: insights from Slovakia. 探索 CJD 发病率趋势：斯洛伐克的启示。

IF 2.3 3区生物学

Prion Pub Date : 2024-12-01 Epub Date: 2024-05-09 DOI: 10.1080/19336896.2024.2349011

Pavol Skacik, Egon Kurca, Stefan Sivak

引用次数: 0

A systemic analysis of Creutzfeldt Jakob disease cases in Asia. 对亚洲克雅氏病病例的系统分析。

IF 2.3 3区生物学

Prion Pub Date : 2024-12-01 Epub Date: 2024-02-07 DOI: 10.1080/19336896.2024.2311950

Urwah Rasheed, Sana Khan, Minahil Khalid, Aneeqa Noor, Saima Zafar

{"title":"A systemic analysis of Creutzfeldt Jakob disease cases in Asia.","authors":"Urwah Rasheed, Sana Khan, Minahil Khalid, Aneeqa Noor, Saima Zafar","doi":"10.1080/19336896.2024.2311950","DOIUrl":"10.1080/19336896.2024.2311950","url":null,"abstract":"Creutzfeldt Jakob Disease (CJD) is a rapidly progressive, fatal neurodegenerative disorder, also known as a subacute spongiform encephalopathy. There are three major subtypes of CJD i.e. Sporadic CJD, which occurs for reasons unbeknown to science (85% of known cases), Genetic or Familial CJD which is characterized by the presence of mutations in the human prion protein (PRNP) gene (10-15% cases) and Iatrogenic CJD that occurs via accidental transmission through medical and surgical procedures (1-2% cases). CJD cases occur globally with 1 case per one million population/year. Considerable data is available related to the incidence and prevalence of CJD in Europe and America. However, the global surveillance database is yet to include Asia even though several Asian countries have their own CJD monitoring units. sCJD is the highest among all CJD cases in Asia. China (1957) and Japan (1705) have reported more cases of sCJD than any Asian country and Hong Kong (1) has reported the least. On the other hand, gCJD is highest in Japan (370) and least in India (2). Our analysis establishes the presence of all variants of CJD across Asia. However, in most Asian countries in general and Southeast Asian countries in particular, CJD cases are misdiagnosed and often underreported. Since Asia is the most populated continent in the world, the actual global prevalence of CJD cannot be estimated until and unless these countries are accounted for. Concrete and reliable surveillance networks are needed across Asia to evaluate the prevalence and incidence of CJD in the region. [Figure: see text]The graphical abstract demonstrates the prevalence of CJD cases in the world and systematically analyses the incidence of CJD in Asian countries between the year 1986-2022. Highest number of cases were reported in Japan followed by China. The study emphasizes the need for assimilation of Asian data in global prevalence.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10854368/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139698853","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

Mutations in human prion-like domains: pathogenic but not always amyloidogenic. 人类朊病毒样结构域的突变：致病但不一定致淀粉样蛋白。

IF 2.3 3区生物学

Prion Pub Date : 2024-12-01 Epub Date: 2024-03-21 DOI: 10.1080/19336896.2024.2329186

Andrea Bartolomé-Nafría, Javier García-Pardo, Salvador Ventura

{"title":"Mutations in human prion-like domains: pathogenic but not always amyloidogenic.","authors":"Andrea Bartolomé-Nafría, Javier García-Pardo, Salvador Ventura","doi":"10.1080/19336896.2024.2329186","DOIUrl":"10.1080/19336896.2024.2329186","url":null,"abstract":"Heterogeneous nuclear ribonucleoproteins (hnRNPs) are multifunctional proteins with integral roles in RNA metabolism and the regulation of alternative splicing. These proteins typically contain prion-like domains of low complexity (PrLDs or LCDs) that govern their assembly into either functional or pathological amyloid fibrils. To date, over 60 mutations targeting the LCDs of hnRNPs have been identified and associated with a spectrum of neurodegenerative diseases including amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), and Alzheimer's disease (AD). The cryo-EM structures of pathological and functional fibrils formed by different hnRNPs have been recently elucidated, including those of hnRNPA1, hnRNPA2, hnRNPDL-2, TDP-43, and FUS. In this review, we discuss the structural features of these amyloid assemblies, placing particular emphasis on scrutinizing the impact of prevalent disease-associated mutations mapping within their LCDs. By performing systematic energy calculations, we reveal a prevailing trend of destabilizing effects induced by these mutations in the amyloid structure, challenging the traditionally assumed correlation between pathogenicity and amyloidogenic propensity. Understanding the molecular basis of this discrepancy might provide insights for developing targeted therapeutic strategies to combat hnRNP-associated diseases.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10962614/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140186323","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

Unmet needs of biochemical biomarkers for human prion diseases. 人类朊病毒疾病对生化生物标志物的需求尚未得到满足。

IF 2.3 3区生物学

Prion Pub Date : 2024-12-01 Epub Date: 2024-05-12 DOI: 10.1080/19336896.2024.2349017

Peter Hermann, Inga Zerr

引用次数: 0

Prion forensics: a multidisciplinary approach to investigate CWD at an illegal deer carcass disposal site. 朊病毒法医学：采用多学科方法调查非法鹿尸处理场的 CWD。

IF 2.3 3区生物学

Prion Pub Date : 2024-12-01 Epub Date: 2024-04-26 DOI: 10.1080/19336896.2024.2343298

Marc D Schwabenlander, Jason C Bartz, Michelle Carstensen, Alberto Fameli, Linda Glaser, Roxanne J Larsen, Manci Li, Rachel L Shoemaker, Gage Rowden, Suzanne Stone, W David Walter, Tiffany M Wolf, Peter A Larsen

引用次数: 0

Correction. 更正。

IF 2.3 3区生物学

Prion Pub Date : 2024-12-01 Epub Date: 2024-05-30 DOI: 10.1080/19336896.2024.2359752

引用次数: 0

Expression of the cellular prion protein by mast cells in white-tailed deer carotid body, cervical lymph nodes and ganglia. 白尾鹿颈动脉体、颈淋巴结和神经节中肥大细胞对细胞朊病毒蛋白的表达。

IF 2.3 3区生物学

Prion Pub Date : 2024-09-16 DOI: 10.1080/19336896.2024.2402225

Anthony E Kincaid,Nathaniel D Denkers,Erin E McNulty,Caitlyn N Kraft,Jason C Bartz,Candace K Mathiason

引用次数: 0

Prion meeting 2023: implications of a growing field 2023 年朊病毒会议：不断发展的领域的影响

IF 2.3 3区生物学

Prion Pub Date : 2024-04-23 DOI: 10.1080/19336896.2024.2343535

T. Outeiro, T. C. R. G. Vieira

{"title":"Prion meeting 2023: implications of a growing field","authors":"T. Outeiro, T. C. R. G. Vieira","doi":"10.1080/19336896.2024.2343535","DOIUrl":"https://doi.org/10.1080/19336896.2024.2343535","url":null,"abstract":"ABSTRACT The history of human prion diseases began with the original description, by Hans Gerhard Creutzfeldt and by Alfons Maria Jakob, of patients with a severe brain disease that included speech abnormalities, confusion, and myoclonus, in a disease that was then named Creutzfeldt Jakob disease (CJD). Later, in Papua New Guinea, a disease characterized by trembling was identified, and given the name “Kuru”. Neuropathological examination of the brains from CJD and Kuru patients, and of brains of sheep with scrapie disease revealed significant similarities and suggested a possible common mode of infection that, at the time, was thought to derive from an unknown virus that caused slow infections. John Stanley Griffith hypothesized that the agent causing these diseases was “probably a protein without nucleic acid” and, in 1982, Stanley Prusiner reported the identification of a proteinaceous infectious particle (coining the term prion) that was resistant to inactivation methods that were at the time standard for nucleic acids, and identified PrP as the major protein component of the infectious agent in scrapie and in Creutzfeldt-Jakob disease, classifying this also as a prion disease. Interestingly, the prion concept had been previously expanded to yeast proteins capable of replicating their conformation, seeding their own aggregation and transmitting phenotypic information. The prion concept has been more recently expanded to refer to misfolded proteins that are capable of converting a normal form of a protein into an abnormal form. The quest to understand and treat prion diseases has united a specific research community around the topic, and regular meetings (Prion Meetings) have taken place over the years to enable discussions, train junior researchers, and inspire research in the field.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140668472","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: 0

A review of chronic wasting disease (CWD) spread, surveillance, and control in the United States captive cervid industry 慢性消耗性疾病（CWD）在美国圈养鹿业的传播、监测和控制回顾

IF 2.3 3区生物学

Prion Pub Date : 2024-04-22 DOI: 10.1080/19336896.2024.2343220

J. Mori, Nelda A Rivera, Jan Novakofski, N. Mateus-Pinilla

{"title":"A review of chronic wasting disease (CWD) spread, surveillance, and control in the United States captive cervid industry","authors":"J. Mori, Nelda A Rivera, Jan Novakofski, N. Mateus-Pinilla","doi":"10.1080/19336896.2024.2343220","DOIUrl":"https://doi.org/10.1080/19336896.2024.2343220","url":null,"abstract":"ABSTRACT Chronic wasting disease (CWD) is a fatal prion disease of the family Cervidae that circulates in both wild and captive cervid populations. This disease threatens the health and economic viability of the captive cervid industry, which raises cervids in contained spaces for purposes such as hunting and breeding. Given the high transmissibility and long incubation period of CWD, the introduction and propagation of the infectious prion protein within and between captive cervid farms could be devastating to individual facilities and to the industry as a whole. Despite this risk, there does not yet exist a literature review that summarizes the scientific knowledge, to date, about CWD spread, surveillance, or control measures. Our review, which focused on peer reviewed, primary research conducted in the United States, sought to address this need by searching Google Scholar, Scopus, and Web of Science with a five-term keyword string containing terms related to the (1) location, (2) species affected, (3) disease, (4) captive cervid industry, and (5) topic of focus. Between the three databases, there were 190 articles that were selected for further examination. Those articles were then read to determine if they were about CWD spread, surveillance, and/or control in captive cervid facilities. The 22 articles that met these inclusion criteria were evaluated in detail and discussed, with recommendations for future collaborative work between captive cervid owners, government agencies, and researchers. This work will help to address, inform, and mitigate the rising problem of CWD spread and establishment.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140674328","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: 0

Novel method for classification of prion diseases by detecting PrPres signal patterns from formalin-fixed paraffin-embedded samples 通过检测福尔马林固定石蜡包埋样本中的 PrPres 信号模式对朊病毒疾病进行分类的新方法

IF 2.3 3区生物学

Prion Pub Date : 2024-04-16 DOI: 10.1080/19336896.2024.2337981

Sachiko Koyama, Kaoru Yagita, Hideomi Hamasaki, Hideko Noguchi, Masahiro Shijo, Kosuke Matsuzono, Kei-Ichiro Takase, Keita Kai, Shin-Ichi Aishima, Kyoko Itoh, Toshiharu Ninomiya, Naokazu Sasagasako, Hiroyuki Honda

引用次数: 0