Early behavioral changes and quantitative analysis of neuropathological features in murine prion disease: stereological analysis in the albino Swiss mice model [0.03%]
小鼠致死性海绵状脑病的行为学改变及神经病理特征的量化分析：瑞士白鼠模型中的立体学研究
Roseane Borner,João Bento-Torres,Diego R V Souza et al.
Roseane Borner et al.
Behavioral and neuropathological changes have been widely investigated in murine prion disease but stereological based unbiased estimates of key neuropathological features have not been carried out. After injections of ME7 infected (ME7) or...
Structural and mechanistic commonalities of amyloid-β and the prion protein [0.03%]
淀粉样蛋白和朊病毒蛋白的结构及机理上的共通性
Bianca Da Costa Dias,Katarina Jovanovic,Danielle Gonsalves et al.
Bianca Da Costa Dias et al.
Amyloid β (Aβ) is a major causative agent of Alzheime disease. This neurotoxic peptide is generated as a result of the cleavage of the Amyloid-Precursor-Protein (APP) by the action of beta secretase and gamma secretase. The neurotoxicity ...
Insoluble cellular prion protein and its association with prion and Alzheimer diseases [0.03%]
不可溶的细胞型朊蛋白及其与朊病和阿尔茨海默病的关系
Wen-Quan Zou,Xiaochen Zhou,Jue Yuan et al.
Wen-Quan Zou et al.
The soluble cellular prion protein (PrP(C)) is best known for its association with prion disease (PrD) through its conversion to a pathogenic insoluble isoform (PrP(Sc)). However, its deleterious effects independent of PrP(Sc) have recently...
RNA-binding proteins with prion-like domains in ALS and FTLD-U [0.03%]
具有朊病毒类似结构域的RNA结合蛋白在ALS和FTLD-U中的作用
Aaron D Gitler,James Shorter
Aaron D Gitler
Amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig's disease) is a debilitating, and universally fatal, neurodegenerative disease that devastates upper and lower motor neurons. The causes of ALS are poorly understood. A central ro...
Ana Lukic,Simon Mead
Ana Lukic
Over the last decade remarkable advances in genotyping and sequencing technology have resulted in hundreds of novel gene associations with disease. These have typically involved high frequency alleles in common diseases and with the advent ...
The p75NTR extracellular domain: a potential molecule regulating the solubility and removal of amyloid-β [0.03%]
p75NTR胞外区是调节淀粉样蛋白可溶性和清除的潜在分子
Xin-Fu Zhou,Yan-Jiang Wang
Xin-Fu Zhou
Senile plaques composed of amyloid-beta (Aβ) in the brain are one of the hallmarks of Alzheimer disease (AD). Removal of Aβ from the brain is the most important therapeutic strategy for AD. The solubility of Aβ is critical for its endocy...
Transmission of prions within the gut and towards the central nervous system [0.03%]
朊病毒在肠道及中枢神经系统的传播
Gianfranco Natale,Michela Ferrucci,Gloria Lazzeri et al.
Gianfranco Natale et al.
The prion protein is a glycoprotein characterized by a folded α-helical structure that, under pathological conditions, misfolds and aggregates into its infectious isoform as β-sheet rich amyloidic deposits. The accumulation of the abnorma...
Alberto Miranda,Eva Pericuesta,Miguel Ángel Ramírez et al.
Alberto Miranda et al.
A large number of studies have analysed the putative functions of the prion protein (PrP(C)) in mammals. Although its sequence conservation over a wide range of different animals may indicate that this protein could have a key role in prion...
The sensitive [SWI (+)] prion: new perspectives on yeast prion diversity [0.03%]
敏感的[SWI+]毒粒：酵母毒粒多样性的新视角
Justin K Hines,Elizabeth A Craig
Justin K Hines
Yeast prions are heritable protein-based genetic elements which rely on molecular chaperone proteins for stable transmission to cell progeny. Within the past few years, five new prions have been validated and 18 additional putative prions i...
(Ctm)PrP and ER stress: a neurotoxic mechanism of some special PrP mutants [0.03%]
变异朊蛋白(PRPS)与内质网应激：某些特殊突变体的神经毒性机制研究
Qi Shi,Xiao-Ping Dong
Qi Shi
The pathogenic agent is hypothesized to be PrP(Sc) in prion diseases. However, little accumulation of PrPSc is repeatedly observed in some kinds of natural and experimental prion diseases, including some special genetic human prion diseases...