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Addressing chronic wasting disease in Korean farms: topsoil removal and 2N NaOH treatment before cervid restocking. 解决韩国农场的慢性消瘦病:在养鱼前去除表土和2N NaOH处理。
IF 1.9 3区 生物学
Prion Pub Date : 2025-12-01 Epub Date: 2025-07-08 DOI: 10.1080/19336896.2025.2527588
Kyung-Je Park, Hoo-Chang Park, Yu-Ran Lee, In-Soon Roh, Gordon Mitchell, Young Pyo Choi, Hyun-Joo Sohn
{"title":"Addressing chronic wasting disease in Korean farms: topsoil removal and 2N NaOH treatment before cervid restocking.","authors":"Kyung-Je Park, Hoo-Chang Park, Yu-Ran Lee, In-Soon Roh, Gordon Mitchell, Young Pyo Choi, Hyun-Joo Sohn","doi":"10.1080/19336896.2025.2527588","DOIUrl":"10.1080/19336896.2025.2527588","url":null,"abstract":"<p><p>Chronic wasting disease (CWD) is a highly contagious prion disease occurring in free-ranging and farmed cervids. In the Republic of Korea, cases of CWD continue to be detected almost annually, on both new and occasionally previously infected farms. CWD-infected animals contaminate soil and other environmental components by shedding prions through their excreta. Since shed prions remain infectious for years in the environment, they can act as infectivity reservoirs facilitating horizontal transmission of CWD. To prevent the further spread of CWD and allow farms to resume operations, control measures on infected farms, including topsoil removal and thorough environmental treatment with 2N NaOH, have been implemented in the Republic of Korea. Restocking remediated farms with cervids was permitted after confirming the absence of prion seeding activity in soil samples using protein misfolding cyclic amplification (PMCA). A total of 215 samples from 18 remediated farms were collected and analysed using PMCA, with only 3 samples from 3 farms displaying prion seeding activity. While the disease control measures effectively eliminated prion seeding activity in CWD-affected farms, CWD recurred at two of the 18 remediated farms 4 to 5 years after restocking animals. It remains unclear whether the recurrence of CWD at the two farms was due to residual prions in the environment after the control measures, or the introduction of the infected animals from other farms. This uncertainty is heightened by the annual occurrence of CWD at multiple farms and the absence of a traceability system for farmed cervids.</p>","PeriodicalId":54585,"journal":{"name":"Prion","volume":"19 1","pages":"20-27"},"PeriodicalIF":1.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12239807/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144585632","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
Prion 2024 conference: from two decades of growth to a new journey forward. 朊病毒2024大会:从二十年成长走向新征程。
IF 1.9 3区 生物学
Prion Pub Date : 2025-12-01 Epub Date: 2025-06-03 DOI: 10.1080/19336896.2025.2514569
Yifei Kong, Pengcheng Huang, Qi Shi, Yifan Wang, Mengting Li, Jiyan Ma, Xiao-Ping Dong, Daojun Hong, Wen-Quan Zou
{"title":"Prion 2024 conference: from two decades of growth to a new journey forward.","authors":"Yifei Kong, Pengcheng Huang, Qi Shi, Yifan Wang, Mengting Li, Jiyan Ma, Xiao-Ping Dong, Daojun Hong, Wen-Quan Zou","doi":"10.1080/19336896.2025.2514569","DOIUrl":"10.1080/19336896.2025.2514569","url":null,"abstract":"<p><p>The Prion 2024 annual conference, held in Nanchang, China, from 23 to 27 October, drew nearly 300 leading scientists, clinicians, researchers, and students from 17 countries to examine the latest advancements in prion research and related diseases. This landmark event marked the inaugural international prion conference hosted in a developing nation for the first time and celebrated the 20th anniversary of the NeuroPrion Association, the organizing body of this prestigious annual gathering - '<i>Celebrating Two Decades of Progress: Pioneering a New Era</i>.' The conference spotlighted key themes such as epidemiology, pathogenesis, the connections between ageing and neurodegenerative diseases. It showcased innovative diagnostic and therapeutic strategies for both human and animal prion diseases, as well as related conditions including Alzheimer's and Parkinson's diseases, prion protein-associated cancers, and renal injury. The programme featured 70 invited talks and 21 selected oral presentations, culminating in 7 plenary sessions led by esteemed speakers, including Nobel Laureate Stanley B. Prusiner. This overview summarizes key presentations and highlights significant aspects of the conference, emphasizing the impactful discussions and collaborations that emerged from this historic event.</p>","PeriodicalId":54585,"journal":{"name":"Prion","volume":"19 1","pages":"1-7"},"PeriodicalIF":1.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144217609","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
Chronic wasting disease as a model for human prion therapy. 慢性消耗性疾病作为人类朊病毒治疗的模型。
IF 1.9 3区 生物学
Prion Pub Date : 2025-12-01 Epub Date: 2025-05-24 DOI: 10.1080/19336896.2025.2510665
Michael Bordonaro
{"title":"Chronic wasting disease as a model for human prion therapy.","authors":"Michael Bordonaro","doi":"10.1080/19336896.2025.2510665","DOIUrl":"10.1080/19336896.2025.2510665","url":null,"abstract":"<p><p>Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are fatal neurodegenerative disorders that result from abnormally folded prion proteins. These disorders can be sporadic, acquired, or genetic. Acquired TSEs can be found in a number of animal species including sheep (scrapie), cows (bovine spongiform encephalopathy), and cervids (chronic wasting disease). Chronic wasting disease (CWD) is unusual in that it is found in free ranging animals in their natural environment. There has been heretofore no reported cases of CWD being transmitted to humans; however, the possibility of future adaption for human transmission exists. Several novel approaches for the prevention and treatment of prion disease in humans are under development, including knockdown of endogenous prion expression or overexpression of dominant negative prion forms. Here, I propose that CWD, as a naturally occurring prion disease, should be considered an important testing target for such therapies, which can demonstrate efficacy outside of controlled laboratory environments. Further, from the ethical standpoint of reducing animal suffering, decreasing the CWD burden in cervid populations is highly desirable. Finally, lowering CWD incidence can reduce future possibilities of transmission to humans or to other animal species.</p>","PeriodicalId":54585,"journal":{"name":"Prion","volume":"19 1","pages":"17-22"},"PeriodicalIF":1.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12118434/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144136600","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
Chronic Wasting Disease Research in North America: A systematic review highlighting species-wise and interdisciplinary research trends. 北美慢性消耗性疾病研究:系统综述,突出物种智慧和跨学科研究趋势。
IF 1.9 3区 生物学
Prion Pub Date : 2025-12-01 Epub Date: 2025-02-17 DOI: 10.1080/19336896.2025.2464753
Chandika Rg, Anaïs Tallon, Emily K Latch
{"title":"Chronic Wasting Disease Research in North America: A systematic review highlighting species-wise and interdisciplinary research trends.","authors":"Chandika Rg, Anaïs Tallon, Emily K Latch","doi":"10.1080/19336896.2025.2464753","DOIUrl":"10.1080/19336896.2025.2464753","url":null,"abstract":"<p><p>Chronic Wasting Disease (CWD) research has experienced significant growth, spanning diverse disciplines such as genetics, immunology, modelling, and behaviour. To gain a broad understanding of the changes in CWD research focusing cervids, we analysed temporal trends in study location, species, genus investigated, infection types, and population type since the discovery of CWD in 1980s. Our findings indicate that Colorado, USA, published the highest number of articles, followed by Wisconsin, and publication numbers correlated with reported CWD cases in states/provinces. <i>Odocoileus</i> emerged as the most studied genus. Wild populations are studied more commonly than captive populations. Keyword analysis of transmission types shows the discovery of novel transmission modes in the recent past. We also used a novel approach to categorize studies into five themes: field-based, lab-based, math/analytics/modelling-based, management-based, and human dimensions. Overall, most studies captured had a lab-based component. The interdisciplinary or transdisciplinary nature of major disciplines and evolving trends in keywords, particularly the increased reliance on genetics/genomics, accentuate the beginning of using genomics to under and tackle CWD at a fundamental scale. Encapsulated in our analysis, these dynamic changes offer valuable insights for navigating CWD through scientifically informed proactive management decisions in conjunction with existing surveillance efforts not only for the commonly studied species but also for potentially susceptible species.</p>","PeriodicalId":54585,"journal":{"name":"Prion","volume":"19 1","pages":"1-16"},"PeriodicalIF":1.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11834482/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143441857","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
Specific early electroencephalogram for the diagnosis of sporadic Creutzfeldt-Jakob disease. 散发性克雅氏病的特异性早期脑电图诊断。
IF 1.9 3区 生物学
Prion Pub Date : 2025-12-01 Epub Date: 2025-03-24 DOI: 10.1080/19336896.2025.2483215
Taiki Matsubayashi, Hirokazu Natsui, Katsuya Satoh, Tetsuyuki Kitamoto, Takanori Yokota, Nobuo Sanjo
{"title":"Specific early electroencephalogram for the diagnosis of sporadic Creutzfeldt-Jakob disease.","authors":"Taiki Matsubayashi, Hirokazu Natsui, Katsuya Satoh, Tetsuyuki Kitamoto, Takanori Yokota, Nobuo Sanjo","doi":"10.1080/19336896.2025.2483215","DOIUrl":"10.1080/19336896.2025.2483215","url":null,"abstract":"<p><p>An early diagnosis is required for intervention in prion disease cases. To elucidate the specificity of early electroencephalography discharges in cases of sporadic Creutzfeldt-Jakob disease, we analysed epileptiform discharges through electroencephalography. Nine patients with methionine/methionine type 1/classic sporadic Creutzfeldt-Jakob disease and 20 patients with status epilepticus were included. Generalized periodic discharges, lateralized periodic discharges, and central sagittal sporadic epileptiform discharges were evaluated. Central sagittal sporadic epileptiform discharges were defined as nonrhythmic and nonperiodic waveforms showing generalized spike-and-wave complexes and/or sharp waves predominantly in the central sagittal region. In the sporadic Creutzfeldt-Jakob disease group, central sagittal sporadic epileptiform discharges, lateralized periodic discharges, and generalized periodic discharges were observed in five (55.6%), one (11.1%), and eight (88.9%) patients, respectively, with an average duration from onset to the appearance of the discharges of 1.6, 1.0, and 2.44 months, respectively. In the status epilepticus group, these discharges were detected in one (5.0%), six (30.0%), and six (30.0%) patients, respectively. The incorporation of central sagittal sporadic epileptiform discharges and lateralized periodic discharges into the World Health Organization diagnostic criteria, alongside generalized periodic discharges, significantly shortened the average lapse from symptom onset to sporadic Creutzfeldt-Jakob disease diagnosis (2.06 months vs. 2.44 months; <i>p</i> = 0.02). Central sagittal sporadic epileptiform discharges emerge as promising biomarkers for distinguishing sporadic Creutzfeldt-Jakob disease from status epilepticus, and together with lateralized periodic discharges provide an opportunity for early diagnosis of sporadic Creutzfeldt-Jakob disease.</p>","PeriodicalId":54585,"journal":{"name":"Prion","volume":"19 1","pages":"17-24"},"PeriodicalIF":1.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11934186/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143694608","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
Functional amyloid protein FXR1 is recruited into neuronal stress granules. 功能性淀粉样蛋白FXR1被招募到神经元应激颗粒中。
IF 1.9 3区 生物学
Prion Pub Date : 2025-12-01 Epub Date: 2025-05-24 DOI: 10.1080/19336896.2025.2505422
Anna A Valina, Tatyana A Belashova, Anastasia K Yuzman, Sergey P Zadorsky, Evgeniy I Sysoev, Vladimir A Mitkevich, Alexander A Makarov, Alexey P Galkin
{"title":"Functional amyloid protein FXR1 is recruited into neuronal stress granules.","authors":"Anna A Valina, Tatyana A Belashova, Anastasia K Yuzman, Sergey P Zadorsky, Evgeniy I Sysoev, Vladimir A Mitkevich, Alexander A Makarov, Alexey P Galkin","doi":"10.1080/19336896.2025.2505422","DOIUrl":"10.1080/19336896.2025.2505422","url":null,"abstract":"<p><p>The FXR1 protein regulates the stability and translation of a number of RNA molecules and plays an important role in the regulation of cellular processes under normal conditions and stress. In particular, this protein is known to be a negative regulator of the key proinflammatory cytokine TNF alpha. We had previously shown that FXR1 functioned in the amyloid form in neurons of the brain of jawed vertebrates. Under stress conditions, FXR1 is incorporated into stress granules in some cell lines, but such studies have not been conducted for neuronal cells. Here, we showed the ability of the FXR1 protein to form cytoplasmic granules in a neuroblastoma cell line under various types of stress. This protein colocalizes with core proteins of neuronal stress granules upon heat shock and sodium arsenite treatment. We also showed that FXR1 colocalizes with anti-amyloid antibodies OC under both normal and stress conditions. Given that stress granules are dynamic structures, we propose that amyloid FXR1-containing RNP particles interact with other stress granule proteins through weak intermolecular hydrogen bonds. Using a yeast model system, we found that FXR1 colocalizes and physically interacts with stress granule proteins such as TIA-1, FMRP, FXR2, and SFPQ. Overall, our results provide new insights into the role of the RNA-binding protein FXR1 in neuronal stress response. We believe that FXR1 inactivation in neuronal stress granules can contribute to an increase in the level of the proinflammatory cytokine TNF alpha in neurodegenerative diseases.</p>","PeriodicalId":54585,"journal":{"name":"Prion","volume":"19 1","pages":"1-16"},"PeriodicalIF":1.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12118398/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144136603","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
Impact of low testing numbers on chronic wasting disease apparent prevalence. 低检测数对慢性消耗性疾病明显患病率的影响。
IF 1.9 3区 生物学
Prion Pub Date : 2025-12-01 Epub Date: 2025-07-10 DOI: 10.1080/19336896.2025.2530534
Jameson J Mori, Nelda A Rivera, William M Brown, Daniel J Skinner, Peter E Schlichting, Jan E Novakofski, Nohra E Mateus-Pinilla
{"title":"Impact of low testing numbers on chronic wasting disease apparent prevalence.","authors":"Jameson J Mori, Nelda A Rivera, William M Brown, Daniel J Skinner, Peter E Schlichting, Jan E Novakofski, Nohra E Mateus-Pinilla","doi":"10.1080/19336896.2025.2530534","DOIUrl":"10.1080/19336896.2025.2530534","url":null,"abstract":"<p><p>Chronic wasting disease (CWD) is a fatal, neurodegenerative disease of cervids, and its management heavily relies on diagnostic testing. Test results are commonly used to calculate 'apparent prevalence' (AP) - the percent of animals tested for CWD (CWD tests) with CWD-positive test results (CWD cases) - but this obscures how tests and cases individually contribute to this statistic. This is most relevant when CWD testing is limited because when few animals are tested, detection of even a single infected deer can result in a high AP that poorly reflects reality. We hypothesized that when CWD testing is limited, AP is negatively driven by testing - rather than cases - with more tests corresponding to lower APs. Graphed CWD surveillance data from townships in Illinois and Wisconsin, USA, indicate that CWD AP values ≥50% were only observed when <23 deer were tested. We used Bayesian multilevel zero-inflated Beta regression to model AP as a function of CWD tests, CWD cases and nonlinear transformations of these two terms separately for each state. The best-fit models of both identified a statistically significant negative relationship between AP and testing numbers that was modified by a positive nonlinear test covariate. This means adding tests when testing is low can have a big impact on decreasing the AP, but this relationship weakens as testing increases. We urge treating apparent prevalences ≥50% with caution and emphasize the importance of increasing the test results when initial surveillance has yielded <23 tests.</p>","PeriodicalId":54585,"journal":{"name":"Prion","volume":"19 1","pages":"28-35"},"PeriodicalIF":1.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12258203/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144610315","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
Detection of chronic wasting disease prions in soil at an illegal white-tailed deer carcass disposal site. 非法白尾鹿尸体处理场土壤中慢性消耗性疾病朊病毒的检测。
IF 1.9 3区 生物学
Prion Pub Date : 2025-12-01 Epub Date: 2025-06-06 DOI: 10.1080/19336896.2025.2514947
Madeline K Grunklee, Stuart S Lichtenberg, Nicole A Lurndahl, Marc D Schwabenlander, Diana L Karwan, E Anu Li, Jason C Bartz, Qi Yuan, Peter A Larsen, Tiffany M Wolf
{"title":"Detection of chronic wasting disease prions in soil at an illegal white-tailed deer carcass disposal site.","authors":"Madeline K Grunklee, Stuart S Lichtenberg, Nicole A Lurndahl, Marc D Schwabenlander, Diana L Karwan, E Anu Li, Jason C Bartz, Qi Yuan, Peter A Larsen, Tiffany M Wolf","doi":"10.1080/19336896.2025.2514947","DOIUrl":"10.1080/19336896.2025.2514947","url":null,"abstract":"<p><p>Chronic wasting disease (CWD) is a contagious prion disorder affecting cervids such as deer, elk, caribou, and moose, causing progressive and severe neurological degeneration followed by eventual death. As CWD prions (PrP<sup>Sc</sup>) accumulate in the body, they are shed through excreta and secreta, as well as through decomposing carcasses. Prions can persist in the environment for years, posing significant concerns for ongoing transmission to susceptible cervids and pose an unknown risk to sympatric species. We used a validated protocol for real-time quaking-induced conversion (RT-QuIC) <i>in vitro</i> prion amplification assay to detect prions in soil collected within and around an illegal white-tailed deer (<i>Odocoileus virginianus</i>, WTD) carcass disposal site and associated captive WTD farm in Beltrami County, Minnesota. We detected PrP<sup>Sc</sup> in 26 of 201 soil samples across 15 locations within the illegal disposal site and one on the farm that housed the cervids. Importantly, a subset of RT-QuIC positive soil samples was collected from soils where carcasses were recovered, providing direct evidence that environmental contamination resulted from this illegal activity. These findings reveal that improper cervid carcass disposal practices may have important implications for ongoing CWD transmission through the environment.</p>","PeriodicalId":54585,"journal":{"name":"Prion","volume":"19 1","pages":"8-19"},"PeriodicalIF":1.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12147482/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144250896","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
Exploring CJD incidence trends: insights from Slovakia. 探索 CJD 发病率趋势:斯洛伐克的启示。
IF 1.9 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
{"title":"Exploring CJD incidence trends: insights from Slovakia.","authors":"Pavol Skacik, Egon Kurca, Stefan Sivak","doi":"10.1080/19336896.2024.2349011","DOIUrl":"10.1080/19336896.2024.2349011","url":null,"abstract":"<p><p>Authors are commenting on the evolving geographical incidence trends observed with the genetic form of Creutzfeldt-Jakob disease and discussing the diverse array of factors contributing to the heightened incidence rates observed in specific geographical regions.</p>","PeriodicalId":54585,"journal":{"name":"Prion","volume":"18 1","pages":"87-88"},"PeriodicalIF":1.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11085948/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140900290","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
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 1.9 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":"<p><p>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.</p>","PeriodicalId":54585,"journal":{"name":"Prion","volume":"18 1","pages":"11-27"},"PeriodicalIF":1.9,"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
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