Prion最新文献_第2页

Prion 2024 Conference Abstracts. Prion 2024 会议摘要。

IF 1.9 3区生物学

Prion Pub Date : 2024-10-01 Epub Date: 2024-11-28 DOI: 10.1080/19336896.2024.2424058

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

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

Expression of the cellular prion protein by mast cells in the human carotid body. 颈动脉肥大细胞表达细胞朊蛋白的研究。

IF 2.3 3区生物学

Prion Pub Date : 2023-12-01 DOI: 10.1080/19336896.2023.2193128

Gregory D Sweetland, Connor Eggleston, Jason C Bartz, Candace K Mathiason, Anthony E Kincaid

{"title":"Expression of the cellular prion protein by mast cells in the human carotid body.","authors":"Gregory D Sweetland, Connor Eggleston, Jason C Bartz, Candace K Mathiason, Anthony E Kincaid","doi":"10.1080/19336896.2023.2193128","DOIUrl":"10.1080/19336896.2023.2193128","url":null,"abstract":"Prion diseases are fatal neurologic disorders that can be transmitted by blood transfusion. The route for neuroinvasion following exposure to infected blood is not known. Carotid bodies (CBs) are specialized chemosensitive structures that detect the concentration of blood gasses and provide feedback for the neural control of respiration. Sensory cells of the CB are highly perfused and densely innervated by nerves that are synaptically connected to the brainstem and thoracic spinal cord, known to be areas of early prion deposition following oral infection. Given their direct exposure to blood and neural connections to central nervous system (CNS) areas involved in prion neuroinvasion, we sought to determine if there were cells in the human CB that express the cellular prion protein (PrPC), a characteristic that would support CBs serving as a route for prion neuroinvasion. We collected CBs from cadaver donor bodies and determined that mast cells located in the carotid bodies express PrPC and that these cells are in close proximity to blood vessels, nerves, and nerve terminals that are synaptically connected to the brainstem and spinal cord.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"17 1","pages":"67-74"},"PeriodicalIF":2.3,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10038025/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9330776","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

Two Chinese patients of sporadic Creutzfeldt-Jacob disease with a S97N mutation in PRNP gene. 伴有PRNP基因S97N突变的中国散发性克雅氏病2例。

IF 2.3 3区生物学

Prion Pub Date : 2023-12-01 Epub Date: 2023-11-14 DOI: 10.1080/19336896.2023.2276921

Dong-Lin Liang, Qi Shi, Kang Xiao, Ruhan A, Wei Zhou, Xiao-Ping Dong

引用次数: 0

Developing neuropalliative care for sporadic Creutzfeldt-Jakob Disease. 发展散发性克雅氏病的神经姑息治疗

IF 1.9 3区生物学

Prion Pub Date : 2022-12-01 DOI: 10.1080/19336896.2022.2043077

Krista L Harrison, Sarah B Garrett, Joni Gilissen, Michael J Terranova, Alissa Bernstein Sideman, Christine S Ritchie, Michael D Geschwind

{"title":"Developing neuropalliative care for sporadic Creutzfeldt-Jakob Disease.","authors":"Krista L Harrison, Sarah B Garrett, Joni Gilissen, Michael J Terranova, Alissa Bernstein Sideman, Christine S Ritchie, Michael D Geschwind","doi":"10.1080/19336896.2022.2043077","DOIUrl":"10.1080/19336896.2022.2043077","url":null,"abstract":"We aimed to identify targets for neuropalliative care interventions in sporadic Creutzfeldt-Jakob disease by examining characteristics of patients and sources of distress and support among former caregivers. We identified caregivers of decedents with sporadic Creutzfeldt-Jakob disease from the University of California San Francisco Rapidly Progressive Dementia research database. We purposively recruited 12 caregivers for in-depth interviews and extracted associated patient data. We analysed interviews using the constant comparison method and chart data using descriptive statistics. Patients had a median age of 70 (range: 60-86) years and disease duration of 14.5 months (range 4-41 months). Caregivers were interviewed a median of 22 (range 11-39) months after patient death and had a median age of 59 (range 45-73) years. Three major sources of distress included (1) the unique nature of sporadic Creutzfeldt-Jakob disease; (2) clinical care issues such as difficult diagnostic process, lack of expertise in sporadic Creutzfeldt-Jakob disease, gaps in clinical systems, and difficulties with end-of-life care; and (3) caregiving issues, including escalating responsibilities, intensifying stress, declining caregiver well-being, and care needs surpassing resources. Two sources of support were (1) clinical care, including guidance from providers about what to expect and supportive relationships; and (2) caregiving supports, including connection to persons with experience managing Creutzfeldt-Jakob disease, instrumental support, and social/emotional support. The challenges and supports described by caregivers align with neuropalliative approaches and can be used to develop interventions to address needs of persons with sporadic Creutzfeldt-Jakob disease and their caregivers.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"16 1","pages":"23-39"},"PeriodicalIF":1.9,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8896185/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42444468","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

Creutzfeldt-Jakob disease after COVID-19: infection-induced prion protein misfolding? A case report. COVID-19后克雅氏病:感染诱导的朊蛋白错误折叠?一份病例报告。

IF 2.3 3区生物学

Prion Pub Date : 2022-12-01 DOI: 10.1080/19336896.2022.2095185

Andrea Bernardini, Gian Luigi Gigli, Francesco Janes, Gaia Pellitteri, Chiara Ciardi, Martina Fabris, Mariarosaria Valente

{"title":"Creutzfeldt-Jakob disease after COVID-19: infection-induced prion protein misfolding? A case report.","authors":"Andrea Bernardini, Gian Luigi Gigli, Francesco Janes, Gaia Pellitteri, Chiara Ciardi, Martina Fabris, Mariarosaria Valente","doi":"10.1080/19336896.2022.2095185","DOIUrl":"https://doi.org/10.1080/19336896.2022.2095185","url":null,"abstract":"Creutzfeldt-Jakob disease (CJD) is a rare, fatal disease presenting with rapidly progressive neurological deficits caused by the accumulation of a misfolded form (PrPSc) of prion protein (PrPc). Coronavirus disease 2019 (COVID-19) is a primarily respiratory syndrome caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2); many diverse neurological complications have been observed after COVID-19. We describe a young patient developing CJD two months after mild COVID-19. Presenting symptoms were visuospatial deficits and ataxia, evolving into a bedridden state with preserved consciousness and diffuse myoclonus. Diagnostic work-up was suggestive of CJD. The early age of onset and the short interval between respiratory and neurological symptoms might suggest a causal relationship: a COVID-19-related neuroinflammatory state may have induced the misfolding and subsequent aggregation of PrPSc. The present case emphasizes the link between neuroinflammation and protein misfolding. Further studies are needed to establish the role of SARS-CoV-2 as an initiator of neurodegeneration.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"16 1","pages":"78-83"},"PeriodicalIF":2.3,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9255144/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10782687","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: 13

Prion therapeutics: Lessons from the past. 朊病毒疗法：过去的教训。

IF 2.3 3区生物学

Prion Pub Date : 2022-12-01 DOI: 10.1080/19336896.2022.2153551

Kyu Hwan Shim, Niti Sharma, Seong Soo A An

{"title":"Prion therapeutics: Lessons from the past.","authors":"Kyu Hwan Shim, Niti Sharma, Seong Soo A An","doi":"10.1080/19336896.2022.2153551","DOIUrl":"10.1080/19336896.2022.2153551","url":null,"abstract":"Prion diseases are a group of incurable zoonotic neurodegenerative diseases (NDDs) in humans and other animals caused by the prion proteins. The abnormal folding and aggregation of the soluble cellular prion proteins (PrPC) into scrapie isoform (PrPSc) in the Central nervous system (CNS) resulted in brain damage and other neurological symptoms. Different therapeutic approaches, including stalling PrPC to PrPSc conversion, increasing PrPSc removal, and PrPC stabilization, for which a spectrum of compounds, ranging from organic compounds to antibodies, have been explored. Additionally, a non-PrP targeted drug strategy using serpin inhibitors has been discussed. Despite numerous scaffolds being screened for anti-prion activity in vitro, only a few were effective in vivo and unfortunately, almost none of them proved effective in the clinical studies, most likely due to toxicity and lack of permeability. Recently, encouraging results from a prion-protein monoclonal antibody, PRN100, were presented in the first human trial on CJD patients, which gives a hope for better future for the discovery of other new molecules to treat prion diseases. In this comprehensive review, we have re-visited the history and discussed various classes of anti-prion agents, their structure, mode of action, and toxicity. Understanding pathogenesis would be vital for developing future treatments for prion diseases. Based on the outcomes of existing therapies, new anti-prion agents could be identified/synthesized/designed with reduced toxicity and increased bioavailability, which could probably be effective in treating prion diseases.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"16 1","pages":"265-294"},"PeriodicalIF":2.3,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9754114/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10657281","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: 4

Difference of geographic distributions of the Chinese patients with prion diseases in the permanent resident places and referring places. 中国朊病毒病患者常住地与转诊地的地理分布差异。

IF 2.3 3区生物学

Prion Pub Date : 2022-12-01 DOI: 10.1080/19336896.2022.2080921

Kang Xiao, Ming-Fan Pang, Yue-Qiao Zhao, Li-Ping Gao, Yue-Zhang Wu, Yuan Wang, Qi Shi, Xiao-Ping Dong

{"title":"Difference of geographic distributions of the Chinese patients with prion diseases in the permanent resident places and referring places.","authors":"Kang Xiao, Ming-Fan Pang, Yue-Qiao Zhao, Li-Ping Gao, Yue-Zhang Wu, Yuan Wang, Qi Shi, Xiao-Ping Dong","doi":"10.1080/19336896.2022.2080921","DOIUrl":"https://doi.org/10.1080/19336896.2022.2080921","url":null,"abstract":"Human prion diseases (PrDs) are a group of transmissible neurodegenerative diseases that can be clarified as sporadic, genetic and iatrogenic forms. In this study, we have analysed the time and geographic distributions of 2011 PrD cases diagnosed by China National Surveillance for Creutzfeldt-Jakob disease (CNS-CJD) since 2006, including 1792 sporadic CJD (sCJD) cases and 219 gPrD cases. Apparently, the cases numbers of both sCJD and gPrD increased along with the surveillance years, showing a stepping up every five years. The geographic distributions of the PrDs cases based on the permanent residences were wide, distributing in 30 out of 31 provincial-level administrative divisions in Chinese mainland. However, the case numbers in the provincial level varied largely. The provinces in the eastern part of China had much more cases than those in the western part. Normalized the case numbers with the total population each province revealed higher incidences in six provinces. Further, the resident and referring places of all PrD cases were analysed, illustrating a clear concentrating pattern of referring in the large metropolises. Five provincial-level administrative divisions reported more PrD cases from other provinces than the local ones. Particularly, BJ reported not only more than one-fourth of all PrDs cases in Chinese mainland but also 3.64-fold more PrDs cases from other provinces than its local ones. We believed that good medical resources, well-trained programmes and knowledge of PrDs in the clinicians and the CDC staffs contributed to well-referring PrD cases in those large cities.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"16 1","pages":"58-65"},"PeriodicalIF":2.3,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9176242/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10393198","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: 1

Variability in prion protein genotypes by spatial unit to inform susceptibility to chronic wasting disease. 朊病毒蛋白基因型在空间单位上的变异，为慢性消耗性疾病的易感性提供信息。

IF 2.3 3区生物学

Prion Pub Date : 2022-12-01 DOI: 10.1080/19336896.2022.2117535

Alberto F Fameli, Jessie Edson, Jeremiah E Banfield, Christopher S Rosenberry, W David Walter

{"title":"Variability in prion protein genotypes by spatial unit to inform susceptibility to chronic wasting disease.","authors":"Alberto F Fameli, Jessie Edson, Jeremiah E Banfield, Christopher S Rosenberry, W David Walter","doi":"10.1080/19336896.2022.2117535","DOIUrl":"https://doi.org/10.1080/19336896.2022.2117535","url":null,"abstract":"Chronic wasting disease (CWD) is a fatal encephalopathy affecting North American cervids. Certain alleles in a host's prion protein gene are responsible for reduced susceptibility to CWD. We assessed for the first time variability in the prion protein gene of elk (Cervus canadensis) present in Pennsylvania, United States of America, a reintroduced population for which CWD cases have never been reported. We sequenced the prion protein gene (PRNP) of 565 elk samples collected over 7 years (2014-2020) and found two polymorphic sites (codon 21 and codon 132). The allele associated with reduced susceptibility to CWD is present in the population, and there was no evidence of deviations from Hardy-Weinberg equilibrium in any of our sampling years (p-values between 0.14 and 1), consistent with the lack of selective pressure on the PRNP. The less susceptible genotypes were found in a frequency similar to the ones reported for elk populations in the states of Wyoming and South Dakota before CWD was detected. We calculated the proportion of less susceptible genotypes in each hunt zone in Pennsylvania as a proxy for their vulnerability to the establishment of CWD, and interpolated these results to obtain a surface representing expected proportion of the less susceptible genotypes across the area. Based on this analysis, hunt zones located in the southern part of our study area have a low proportion of less susceptible genotypes, which is discouraging for elk persistence in Pennsylvania given that these hunt zones are adjacent to the deer Disease Management Area 3, where CWD has been present since 2014.","PeriodicalId":54585,"journal":{"name":"Prion","volume":"16 1","pages":"254-264"},"PeriodicalIF":2.3,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9481152/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10421581","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