IF 1.6 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":1.6,"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 1.6 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":"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.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"19 1","pages":"28-35"},"PeriodicalIF":1.6,"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 1.7 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":"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 (PrPSc) 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) in vitro prion amplification assay to detect prions in soil collected within and around an illegal white-tailed deer (Odocoileus virginianus, WTD) carcass disposal site and associated captive WTD farm in Beltrami County, Minnesota. We detected PrPSc 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.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"19 1","pages":"8-19"},"PeriodicalIF":1.7,"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

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

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 1.9 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

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