Omics of Prion Diseases [0.03%]
朊毒病的组学研究进展
Silvia Vanni
Silvia Vanni
Prion diseases are unique neurodegenerative pathologies that can occur with sporadic, genetic, and acquired etiologies. Human and animal prion diseases can be recapitulated in laboratory animals with good reproducibility providing highly co...
Michele Fiorini,Matilde Bongianni,Salvatore Monaco et al.
Michele Fiorini et al.
Prion disease or transmissible spongiform encephalopathies are characterized by the presence of the abnormal form of the prion protein (PrPSc). The pathological and transmissible properties of PrPSc are enciphered in its secondary and terti...
Amplified Detection of Prions and Other Amyloids by RT-QuIC in Diagnostics and the Evaluation of Therapeutics and Disinfectants [0.03%]
RT-QuIC在诊断、治疗评估和消毒剂评估中放大检测朊病毒和其他淀粉样蛋白的作用
Byron Caughey,Christina D Orru,Bradley R Groveman et al.
Byron Caughey et al.
Among the most sensitive, specific and practical of methods for detecting prions are the real-time quaking-induced conversion (RT-QuIC) assays. These assays exploit the fundamental self-propagating activity of prions to amplify the presence...
Fabio Moda
Fabio Moda
Transmissible spongiform encephalopathies, or prion diseases, are a group of incurable disorders caused by the accumulation of an abnormally folded prion protein (PrPSc) in the brain. According to the "protein-only" hypothesis, PrPSc is the...
Copper- and Zinc-Promoted Interdomain Structure in the Prion Protein: A Mechanism for Autoinhibition of the Neurotoxic N-Terminus [0.03%]
铜和锌促进的朊病毒蛋白分子间结构：神经毒性N端自抑制机制的一种解释
Eric G B Evans,Glenn L Millhauser
Eric G B Evans
The function of the cellular prion protein (PrPC), while still poorly understood, is increasingly linked to its ability to bind physiological metal ions at the cell surface. PrPC binds divalent forms of both copper and zinc through its unst...
Jesús R Requena,Holger Wille
Jesús R Requena
The prion diseases, which include Creutzfeldt-Jakob disease in humans, chronic wasting disease in cervids (i.e., deer, elk, moose, and reindeer), bovine spongiform encephalopathy in cattle, as well as sheep and goat scrapie, are caused by t...
Diane L Ritchie,James W Ironside
Diane L Ritchie
The human prion diseases comprise sporadic, genetic, and acquired disorders. These are rare conditions with a heterogeneous clinicopathologic phenotype, which can make diagnosis challenging. A combined clinical, genetic, neuropathologic and...
Richard Knight
Richard Knight
Prion diseases are progressive fatal encephalopathies characterized by a neurodegenerative pathology, the tissue deposition of abnormally folded prion protein and, in general, potential transmissibility. Creutzfeldt-Jakob disease (CJD) is t...
Candace K Mathiason
Candace K Mathiason
Transmissible spongiform encephalopathies (TSEs), or prions, are neurodegenerative diseases that affect a variety of animal species, including humans. Cruetzfeldt-Jakob disease (CJD) in humans, sheep and goat scrapie, chronic wasting diseas...
Phenotypical Variability in Bovine Spongiform Encephalopathy: Epidemiology, Pathogenesis, and Diagnosis of Classical and Atypical Forms [0.03%]
牛海绵状脑病的表型变异：经典型和非常规型暴发、致病机理及诊断方法的研究
Cristiano Corona,Elena Vallino Costassa,Barbara Iulini et al.
Cristiano Corona et al.
After thirty years, bovine spongiform encephalopathy (BSE) still represents the biggest crisis in the field of food safety. Initially detected in the United Kingdom in 1986, BSE spread to many other countries all over the world, involving a...