Asen Daskalov,Sven J Saupe
Asen Daskalov
Formation of higher-order supramolecular complexes has emerged as a common principle underlying activity of a number of immune and regulated cell-death signalling pathways in animals, plants and fungi. Some of these signalosomes employ func...
System degeneration in an MM1-type sporadic Creutzfeldt-Jakob disease case with an unusually prolonged akinetic mutism state [0.03%]
一例特殊类型的散发性克雅病的系统退变：持续僵住状态时间异常延长的MM1型克雅病病例报告
Yasushi Iwasaki,Keiko Mori,Masumi Ito et al.
Yasushi Iwasaki et al.
Methionine/methionine type 1 (MM1-type) sporadic Creutzfeldt-Jakob disease (sCJD), known as the 'classic type,' shows typical clinicopathological sCJD findings. In general, patients reach an akinetic mutism state within a few months of dise...
Case Reports
Prion. 2021 Dec;15(1):12-20. DOI:10.1080/19336896.2020.1868931 2021
Exploration of genetic factors resulting in abnormal disease in cattle experimentally challenged with bovine spongiform encephalopathy [0.03%]
牛海绵状脑病实验感染家畜异常疾病遗传因素的研究
Sandor Dudas,Renee Anderson,Antanas Staskevicus et al.
Sandor Dudas et al.
Since the discovery of bovine spongiform encephalopathy (BSE), researchers have orally challenged cattle with infected brain material to study various aspects of disease pathogenesis. Unlike most other pathogens, oral BSE challenge does not...
On the role of the cellular prion protein in the uptake and signaling of pathological aggregates in neurodegenerative diseases [0.03%]
细胞型朊蛋白在神经退行性疾病中病理聚集体摄取和信号转导中的作用
Giuseppe Legname,Carlo Scialò
Giuseppe Legname
Neurodegenerative disorders are associated with intra- or extra-cellular deposition of aggregates of misfolded insoluble proteins. These deposits composed of tau, amyloid-β or α-synuclein spread from cell to cell, in a prion-like manner. ...
Involvement of N- and C-terminal region of recombinant cervid prion protein in its reactivity to CWD and atypical BSE prions in real-time quaking-induced conversion reaction in the presence of high concentrations of tissue homogenates [0.03%]
鹿源重组prP N-和C-端结构区与高浓度组织匀浆共孵育后参与异常山羊PrPSc和CWD Sc致病性反应的实验研究
Akio Suzuki,Kazuhei Sawada,Takeshi Yamasaki et al.
Akio Suzuki et al.
The real-time quaking-induced conversion (RT-QuIC) reaction is a sensitive and specific method for detecting prions. However, inhibitory factors present in tissue homogenates can easily interfere with this reaction. To identify the RT-QuIC ...
Experimental oral transmission of chronic wasting disease to sika deer (Cervus nippon) [0.03%]
慢性消耗性疾病经口感染梅花鹿试验研究报告
Hyun-Joo Sohn,Gordon Mitchell,Yoon Hee Lee et al.
Hyun-Joo Sohn et al.
Chronic wasting disease (CWD) affects a broad array of cervid species and continues to be detected in an expanding geographic range. Initially introduced into the Republic of Korea through the importation of CWD-infected elk (Cervus canaden...
Search for functional amyloid structures in chicken and fruit fly female reproductive cells [0.03%]
家禽和果蝇雌性生殖细胞中功能性淀粉样蛋白结构的研究
V A Siniukova,J V Sopova,S A Galkina et al.
V A Siniukova et al.
We conducted a cytological search for amyloid structures in female reproductive cells of Gallus gallus domesticus and Drosophila melanogaster. We have shown that the amyloid-specific dye, Thioflavin S, but not Congo red, stains some cytopla...
RT-QuIC detection of tauopathies using full-length tau substrates [0.03%]
使用全长tau底物检测tau蛋白病的RT-QuIC技术
Joanne M Tennant,Davin M Henderson,Thomas M Wisniewski et al.
Joanne M Tennant et al.
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 d...
Age structuring and spatial heterogeneity in prion protein gene (PRNP) polymorphism in white-tailed deer [0.03%]
白尾鹿中朊病毒蛋白基因（PRNP）多态性的年龄结构和空间异质性
Tyler K Chafin,Marlis R Douglas,Bradley T Martin et al.
Tyler K Chafin et al.
Chronic-wasting disease (CWD) is a prion-derived fatal neurodegenerative disease that has affected wild cervid populations on a global scale. Susceptibility has been linked unambiguously to several amino acid variants within the prion prote...
Corticobasal manifestations of Creutzfeldt-Jakob disease with D178N-homozygous 129M genotype [0.03%]
具有D178N-同源129M基因型的克罗伊茨费尔特雅各布病的皮质基底表现形式
Yumeng Huang,Ma Jianfang,Rodrigo Morales et al.
Yumeng Huang et al.
Creutzfeldt-Jakob disease (CJD) is a prion disease, usually presented with memory loss, ataxia, dementia, myoclonus, involuntary movements and psychiatric problems. D178N-homozygous 129M genotype has been recognized in the diagnosis of fata...
Case Reports
Prion. 2020 Dec;14(1):232-237. DOI:10.1080/19336896.2020.1812367 2020