Prion最新文献 - Book学术

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":"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.","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":"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. Odocoileus 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.","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":"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; p = 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.","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":"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.","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

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

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":"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":"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

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 1.9 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":"18 1","pages":"28-39"},"PeriodicalIF":1.9,"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

A case report of fatal familial insomnia with cerebrospinal fluid leukocytosis during the COVID-19 epidemic and review of the literature. COVID-19 流行期间致命性家族性失眠伴脑脊液白细胞增多的病例报告及文献综述。

IF 1.9 3区生物学

Prion Pub Date : 2024-12-01 Epub Date: 2024-01-16 DOI: 10.1080/19336896.2023.2298520

Zheng Wang, Yueqi Huang, Shuqi Wang, Jiefang Chen, Gesang Meiduo, Man Jin, Xiaoying Zhang

{"title":"A case report of fatal familial insomnia with cerebrospinal fluid leukocytosis during the COVID-19 epidemic and review of the literature.","authors":"Zheng Wang, Yueqi Huang, Shuqi Wang, Jiefang Chen, Gesang Meiduo, Man Jin, Xiaoying Zhang","doi":"10.1080/19336896.2023.2298520","DOIUrl":"10.1080/19336896.2023.2298520","url":null,"abstract":"Fatal familial insomnia (FFI) is a rare autosomal dominant genetic neurodegenerative disease. Generally, FFI patients will develop rapidly progressive dementia, sleep disturbance, autonomic dysfunction, and so on. Cerebrospinal fluid examination of FFI patients normally shows no obvious abnormalities. Here, we report a young male patient who was diagnosed with FFI during the COVID-19 epidemic. Clinical symptoms include psychobehavioral abnormality, cognitive decline, sleep disturbance, and autonomic dysfunction. No abnormalities were found in routine examinations after admission. However, the number of white blood cells in the cerebrospinal fluid increased. Though the patient was treated with anti-infection and immunotherapy, the symptoms were not relieved. A lumbar puncture was performed again, and it was found that the total Tau protein in the cerebrospinal fluid was elevated, and PET results showed that brain metabolism decreased. Finally, a genetic test was used to confirm the diagnosis of FFI. This case suggests that patients with FFI may also have elevated white blood cells in cerebrospinal fluid and timely detection of Tau protein in cerebrospinal fluid is helpful for early identification of FFI. And precise diagnosis relies on genetic testing.","PeriodicalId":54585,"journal":{"name":"Prion","volume":" ","pages":"1-10"},"PeriodicalIF":1.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11654706/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139472700","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 1.9 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

Correction. 更正。

IF 1.9 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