解剖学和形态学
麻醉学
听力与言语-语言病理学
行为科学
心脏和心血管系统
细胞和组织工程学
临床神经病学
危重症监护医学
牙科,口腔外科和医学
皮肤病学
急诊医学
内分泌学和新陈代谢
肠胃学和肝脏学
老人病学和老年医学
卫生保健科学和服务
血液学
免疫学
传染病
综合和补充性医学
医学伦理学
医学信息学
医学实验室技术
医学,全科和内科
医学,法律
医学,研究和试验
神经系统科学
护理
营养学和饮食学
产科医学和妇科医学
肿瘤学
眼科学
整形外科学
耳鼻喉科学
病理学
儿科学
周围血管疾病
药理学和药剂学
生理学
基本医疗保健
精神病学
公共、环境和职业卫生
放射学,核医学和医学成像
康复学
生殖生物学
呼吸系统
风湿病学
运动科学
外科学
毒理学
热带医学
泌尿学和肾脏学
病毒学
老年医学
健康政策和服务
心理学,临床
abstract::Early detection and diagnosis of neurodegenerative diseases has been hampered by the lack of sensitive testing. Real-time quaking induced conversion (RT-QuIC) has been used for the early and sensitive detection of prion-induced neurologic disease, and has more recently been adapted to detect misfolded alpha-synuclein ...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2020.1832946
更新日期:2020-12-01 00:00:00
abstract::The description of prions as causal agents of Transmissible Spongiform Encephalopathies (TSE), is nowadays accepted as an important breakthrough in biology as revealed the existence of a completely new group of pathogens and a new way of transmission for biological information. A common feature of many neurodegenerati...
journal_title:Prion
pub_type:
doi:10.1080/19336896.2019.1569451
更新日期:2019-01-01 00:00:00
abstract:OBJECTIVE:To evaluate whether EPC-MVs could promote bone regeneration by directly regulating osteoblast through miR-126. The underlying mechanisms were also explored. METHODS:EPCs were isolated from bone marrow mononuclear cells. EPC-MVs were collected from EPCs cultured medium. The lentivirus was used to induce miR-1...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2019.1607464
更新日期:2019-01-01 00:00:00
abstract::TDP-43 and FUS are DNA/RNA binding proteins associated with neuronal inclusions in amyotrophic lateral sclerosis (ALS) patients. Other neurodegenerative diseases are also characterized by neuronal protein aggregates, e.g. Huntington's disease, associated with polyglutamine (polyQ) expansions in the protein huntingtin....
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2017.1423185
更新日期:2018-01-02 00:00:00
abstract::Studies of the ovine prion-related protein (testis-specific) gene (PRNT), including studies of genetic diversity, have highlighted its potential relationship to scrapie infection and economically important ovine traits. PRNT was previously reported to be highly polymorphic in Portuguese sheep. To characterize genetic ...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2018.1467193
更新日期:2018-01-01 00:00:00
abstract::Transmissible spongiform encephalopathies (TSEs) are a group of lethal neurodegenerative diseases involving the structural conversion of cellular prion protein (PrPC) into the pathogenic isoform (PrPSc) for which no effective treatment is currently available. Previous studies have implicated that a polymeric molecule ...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2018.1505395
更新日期:2018-01-01 00:00:00
abstract::M2B cells with persistent classical bovine spongiform encephalopathy (C-BSE) have been established previously. In this study, we performed strain characterization of the M2B cell line in bovine PrPC overexpressing mice (Tg 1896). Mice intracranially inoculated with M2B cells and C-BSE survived for 451 ± 7 and 465 ± 31...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2017.1331809
更新日期:2017-11-02 00:00:00
abstract::The normal function of PrPC, the cellular prion protein, has remained mysterious since its first description over 30 years ago. Amazingly, although complete deletion of the gene encoding PrPC has little phenotypic consequence, expression in transgenic mice of PrP molecules carrying certain internal deletions produces ...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2017.1384894
更新日期:2017-11-02 00:00:00
abstract::In most human and animal prion diseases the abnormal disease-associated prion protein (PrPSc) is deposited as non-amyloid aggregates in CNS, spleen and lymphoid organs. In contrast, in humans and transgenic mice with PrP mutations which cause expression of PrP lacking a glycosylphosphatidylinositol (GPI)-anchor, most ...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2017.1336274
更新日期:2017-07-04 00:00:00
abstract::The prion paradigm is increasingly invoked to explain the molecular pathogenesis of neurodegenerative diseases involving the misfolding and aggregation of proteins other than the prion protein (PrP). Extensive evidence from in vitro and in vivo studies indicates that misfolded and aggregated Aβ peptide, which is the p...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2017.1334029
更新日期:2017-07-04 00:00:00
abstract::Modified nucleosides in tRNA anticodon loops such as 5-methoxy-carbonyl-methyl-2-thiouridine (mcm5s2U) and pseuduridine (Ψ) are thought to be required for an efficient decoding process. In Saccharomyces cerevisiae, the simultaneous presence of mcm5s2U and Ψ38 in tRNAGlnUUG was shown to mediate efficient synthesis of t...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2017.1284734
更新日期:2017-01-02 00:00:00
abstract::Conversion of native cellular prion protein (PrPc) from an α-helical structure to a toxic and infectious β-sheet structure (PrPSc) is a critical step in the development of prion disease. There are some indications that the formation of PrPSc is preceded by a β-sheet rich PrP (PrPβ) form which is non-infectious, but is...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2016.1254857
更新日期:2016-11-01 00:00:00
abstract::Recent studies on iatrogenic Creutzfeldt-Jakob disease (CJD) raised concerns that one of the hallmark lesions of Alzheimer disease (AD), amyloid-β (Aβ), may be transmitted from human-to-human. The neuropathology of AD-related lesions is complex. Therefore, many aspects need to be considered in deciding on this issue. ...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.1080/19336896.2016.1228507
更新日期:2016-09-02 00:00:00
abstract::The detection method based on the mathematical expectation (ME) strategy is fast and accuracy for low frequency mutation screening in large samples. Previous studies have found that the 14-bp insertion/deletion (indel) variants of the 3' untranslated region (3' UTR) within bovine PRNP gene have been characterized with...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2016.1211593
更新日期:2016-09-02 00:00:00
abstract::Tauopathies are a family of neurodegenerative diseases in which fibrils of human hyperphosphorylated tau (P-tau) are believed to cause neuropathology. In Alzheimer disease, P-tau associates with A-beta amyloid and contributes to disease pathogenesis. In familial human prion diseases and variant CJD, P-tau often co-ass...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2016.1199313
更新日期:2016-07-03 00:00:00
abstract::Bovine spongiform encephalopathy (BSE) created a global European crisis in the 1980s and 90s, with very serious health and economic implications. Classical BSE now appears to be under control, to a great extent as a result of a global research effort that identified the sources of prions in meat and bone meal (MBM) an...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2016.1175801
更新日期:2016-05-03 00:00:00
abstract::Proteins with expanded polyglutamine (polyQ) regions are prone to form amyloids, which can cause diseases in humans and toxicity in yeast. Recently, we showed that in yeast non-toxic amyloids of Q-rich proteins can induce aggregation and toxicity of wild type huntingtin (Htt) with a short non-pathogenic polyglutamine ...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2016.1176659
更新日期:2016-05-03 00:00:00
abstract::Genetic prion diseases are degenerative brain disorders caused by mutations in the gene encoding the prion protein (PrP). Different PrP mutations cause different diseases, including Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome and fatal familial insomnia (FFI). The reason for this var...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2016.1139276
更新日期:2016-03-03 00:00:00
abstract::Among different types of protein aggregation, amyloids are a biochemically well characterized state of protein aggregation that are associated with a large number of neurodegenerative diseases including Parkinson's disease, Alzheimer and Creutzfeldt-Jakob disease. Yeast, Saccharomyces cerevisiae is an insightful model...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2016.1141858
更新日期:2016-03-03 00:00:00
abstract::Transmissible spongiform encephalopathies (TSEs) are a group of neurodegenerative diseases caused by the misfolding of the cellular prion protein to an infectious form PrP(Sc). The intercellular transfer of PrP(Sc) is a question of immediate interest as the cell-to-cell movement of the infectious particle causes the i...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2015.1025189
更新日期:2015-01-01 00:00:00
abstract::A variety of signaling pathways, in particular with roles in cell fate and host defense, operate by a prion-like mechanism consisting in the formation of open-ended oligomeric signaling complexes termed signalosomes. This mechanism emerges as a novel paradigm in signal transduction. Among the proteins forming such sig...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.1080/19336896.2015.1038018
更新日期:2015-01-01 00:00:00
abstract::Mammalian prions are composed of misfolded aggregated prion protein (PrP) with amyloid-like features. Prions are zoonotic disease agents that infect a wide variety of mammalian species including humans. Mammals and by-products thereof which are frequently encountered in daily life are most important for human health. ...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2015.1065373
更新日期:2015-01-01 00:00:00
abstract::The objectives of this study were to test whether recombinant mouse (mo)PrP alone or in combination with LPS or under simulated endotoxemia would affect expression of genes related to host inflammatory and antimicrobial responses. To test our hypotheses colon tissues were collected from 16 male mice (FVB/N strain) and...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2015.1019694
更新日期:2015-01-01 00:00:00
abstract::Prion protein, PrP(C), is a glycoprotein that is expressed on the cell surface beginning with the early stages of embryonic stem cell differentiation. Previously, we showed that ectopic expression of PrP(C) in human embryonic stem cells (hESCs) triggered differentiation toward endodermal, mesodermal, and ectodermal li...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.32079
更新日期:2014-01-01 00:00:00
abstract::The conformational diseases, linked to protein aggregation into amyloid conformations, range from non-infectious neurodegenerative disorders, such as Alzheimer disease (AD), to highly infectious ones, such as human transmissible spongiform encephalopathies (TSEs). They are commonly known as prion diseases. However, si...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/19336896.2014.968464
更新日期:2014-01-01 00:00:00
abstract::Abstract Here, we reported a Chinese case of Creutzfeldt-Jakob disease (CJD) with a rare mutation in the prion protein gene (PRNP) leading to an exchange of amino acid from valine (V) to isoleucine (I) at codon 180 (V180I). The 72 year-old Chinese women started with gradual memory loss. On admission, she did not prese...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/19336896.2014.967040
更新日期:2014-01-01 00:00:00
abstract::Prion and Alzheimer diseases are fatal neurodegenerative diseases caused by misfolding and aggregation of the cellular prion protein (PrP(C)) and the β-amyloid peptide, respectively. Soluble oligomeric species rather than large aggregates are now believed to be neurotoxic. PrP(C) undergoes three proteolytic cleavages ...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.27438
更新日期:2014-01-01 00:00:00
abstract::Prions, the causative agent of chronic wasting disease (CWD) enter the environment through shedding of bodily fluids and carcass decay, posing a disease risk as a result of their environmental persistence. Plants have the ability to take up large organic particles, including whole proteins, and microbes. This study us...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.27963
更新日期:2014-01-01 00:00:00
abstract::Prion diseases are consistently associated with prion protein (PrP(C)) misfolding rendering a cascade of auto-catalytic self-perpetuation of misfolded PrP in an afflicted individual. The molecular process is intriguingly similar to all known amyloid diseases both local and systemic. The prion disease is also infectiou...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.27601
更新日期:2014-01-01 00:00:00
abstract::The pathogenic mechanism of prion diseases remains unknown. We recently reported that prion infection disturbs post-Golgi trafficking of certain types of membrane proteins to the cell surface, resulting in reduced surface expression of membrane proteins and abrogating the signal from the proteins. The surface expressi...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.27381
更新日期:2013-11-01 00:00:00
abstract::The molecular chaperone network plays a critical role in the formation and propagation of self-replicating yeast prions. Not only do individual prions differ in their requirements for certain chaperones, but structural variants of the same prion can also display distinct dependences on the chaperone machinery, specifi...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.26547
更新日期:2013-09-01 00:00:00
abstract::Many laboratory studies and epidemiological observations confirm that nematodes prevent some immune-mediated diseases. The development of immunologically well-defined laboratory models of intestinal nematode infection has allowed significant advances to be made in understanding the immunological basis of effector mech...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.25008
更新日期:2013-07-01 00:00:00
abstract::Mechanisms for the spread of transmissible spongiform encephalopathy diseases, including chronic wasting disease (CWD) in North American cervids, are incompletely understood, but primary routes include horizontal and environmental transmission. Birds have been identified as potential vectors for a number of diseases, ...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.25621
更新日期:2013-07-01 00:00:00
abstract::Here, we report a Chinese case of Creutzfeldt-Jakob disease (CJD) with a rare mutation in the prion protein gene (PRNP) leading to an exchange of amino acid from valine (Val) to isoleucine (I) at codon 203 (V203I). The 80-y-old male presented with sudden memory loss, rapid loss of vocabulary, inattention and slow resp...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.24674
更新日期:2013-05-01 00:00:00
abstract::The degeneration of pre-synaptic boutons in the stratum radiatum of the dorsal hippocampus is one of earliest components of neurodegeneration in several models of murine prion disease. We recently showed that blockade of synaptic transmission by infusion of botulinum neurotoxin A (BoNT/A) into the hippocampus several ...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.23327
更新日期:2013-03-01 00:00:00
abstract::Growing evidence indicates that astrocytes cannot be just considered as passive supportive cells deputed to preserve neuronal activity and survival, but rather they are involved in a striking number of active functions that are critical to the performance of the central nervous system (CNS). As a consequence, it is be...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.22512
更新日期:2013-01-01 00:00:00
abstract::Shadoo (Sho) is a brain glycoprotein with similarities to the unstructured region of PrP (C) . Frameshift alleles of the Sho gene, Sprn, are reported in variant Creutzfeldt-Jakob disease (vCJD) patients while Sprn mRNA knockdown in PrP-null (Prnp(0/0) ) embryos produces lethality, advancing Sho as the hypothetical PrP...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.21867
更新日期:2012-11-01 00:00:00
abstract::It has been described that the breakdown of β-sheets in PrP (Sc) by denaturation results in loss of infectivity and PK-sensitivity, suggesting a relationship between the structure and PK-resistance. It is also known that an important fraction of total PrP (Sc) is PK-sensitive and can be isolated by the method we alr...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.22309
更新日期:2012-11-01 00:00:00
abstract::Each known abnormal prion protein (PrP (Sc) ) is considered to have a specific range and therefore the ability to infect some species and not others. Consequently, some species have been assumed to be prion disease resistant as no successful natural or experimental challenge infections have been reported. This assumpt...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.22057
更新日期:2012-11-01 00:00:00
abstract::The evolutionary origins of vertebrate prion genes had remained elusive until recently when multiple lines of evidence converged to the proposition that members of the prion gene family represent an ancient branch of a larger family of ZIP metal ion transporters. (1) A follow-up investigation which explored the mechan...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.20196
更新日期:2012-09-01 00:00:00