Christina J Sigurdson,Jason C Bartz,K Peter R Nilsson
Christina J Sigurdson
Amyloid fibrils share a structural motif consisting of highly ordered β-sheets aligned perpendicular to the fibril axis ( 1, 2) . At each fibril end, β-sheets provide a template for recruiting and converting monomers ( 3) . Various amyloi...
A Chinese Creutzfeldt-Jakob disease patient with E196K mutation in PRNP [0.03%]
中国发现朊病毒病患者PRNP基因E196K突变
Qi Shi,Cao Chen,Xiao-Nan Song et al.
Qi Shi et al.
Genetic Creutzfeldt-Jakob disease (gCJD) is caused by a range of mutations in the prion protein gene (PRNP) and account for approximately 10-15% of overall human prion diseases worldwide. They are different with disease onset, disease durat...
Case Reports
Prion. 2011 Apr-Jun;5(2):117-20. DOI:10.4161/pri.5.2.15846 2011
Characterization of Syrian hamster adapted prions derived from L-type and C-type bovine spongiform encephalopathies [0.03%]
叙利亚仓鼠适应型L素和C素牛海绵状脑病朊病毒的特征分析
Yujing Shu,Kentaro Masujin,Hiroyuki Okada et al.
Yujing Shu et al.
Atypical forms of bovine spongiform encephalopathy (BSE) may be caused by different prions from classical BSE (C-BSE). In this study, we examined the susceptibility of mice overexpressing mouse and hamster chimeric prion protein (PrP) to L-...
Jimmy Savistchenko,Zaira E Arellano-Anaya,Olivier Andréoletti et al.
Jimmy Savistchenko et al.
Protein misfolding is central to the pathogenesis of several neurodegenerative disorders. Among these disorders, prion diseases are unique because they are transmissible. The conversion of the host-encoded GPI-anchored PrP protein into a st...
Disulfide mapping reveals the domain swapping as the crucial process of the structural conversion of prion protein [0.03%]
二硫键定位揭示了结构转换过程中瘙痒病蛋白关键的域交换过程
Iva Hafner-Bratkovič,Roman Jerala
Iva Hafner-Bratkovič
Prion diseases are infectious conformational diseases. Despite the determination of many native prion protein (PrP) structures and in vitro production of infectious prions from recombinant PrP the structural background of PrP conversion rem...
Establishment and characterization of Prnp knockdown neuroblastoma cells using dual microRNA-mediated RNA interference [0.03%]
利用双microRNA介导的RNA干扰建立和鉴定Prnp低表达神经母细胞瘤细胞系
Sang-Gyun Kang,Yu-Mi Roh,Agnes Lau et al.
Sang-Gyun Kang et al.
Prion diseases are fatal transmissible neurodegenerative disorders. In the pathogenesis of the disease, the cellular prion protein (PrPC) is required for replication of abnormal prion (PrPSc), which results in accumulation of PrPSc. Althoug...
RepA-WH1 prionoid: a synthetic amyloid proteinopathy in a minimalist host [0.03%]
最小化宿主中的合成淀粉样蛋白病：RepA-WH1朊病毒样蛋白质异常聚集体
Rafael Giraldo,Susana Moreno-Díaz de la Espina,M Elena Fernández-Tresguerres et al.
Rafael Giraldo et al.
The intricate complexity, at the molecular and cellular levels, of the processes leading to the development of amyloid proteinopathies is somehow counterbalanced by their common, universal structural basis. The later has fueled the quest fo...
Charles E Mays,Chongsuk Ryou
Charles E Mays
The biochemical essence of prion replication is the molecular multiplication of the disease-associated misfolded isoform of prion protein (PrP), termed PrPSc, in a nucleic acid-free manner. PrP(Sc) is generated by the protein misfolding pro...
Dementia screening, biomarkers and protein misfolding: Implications for public health and diagnosis [0.03%]
痴呆筛查、生物标志物和蛋白质错误折叠：对公共卫生和诊断的意义
James E Galvin
James E Galvin
Misfolded proteins are at the core of many neurodegenerative diseases, nearly all of them associated with cognitive impairment. For example Creutzfeldt-Jacob disease is associated with aggregation of prion protein, Lewy body dementia and Pa...
Gianluigi Forloni,Claudia Balducci
Gianluigi Forloni
The relationship between Alzheimer disease (AD) and prion-related encephalopathies (TSE) has been proposed by different points of view. Recently, the scientific attention has been attracted by the results proposing the possibility that PrPc...