Insight into the conserved structural dynamics of the C-terminus of mammal PrPC identifies structural core and possible structural role of pharmacological chaperones [0.03%]
保守的结构动力学见解发现了哺乳动物PrPC尾部结构核心及药理增容剂的可能结构作用
Patricia Soto,Garrett M Gloeb,Kaitlin A Tsuchida et al.
Patricia Soto et al.
Misfolding of the prion protein is central to prion disease aetiology. Although understanding the dynamics of the native fold helps to decipher the conformational conversion mechanism, a complete depiction of distal but coupled prion protei...
A family with mental disorder as the first symptom finally confirmed with Gerstmann-Sträussler-Scheinker disease with P102L mutation in PRNP gene - case report [0.03%]
以精神病为主要表现的家族性Gerstmann-Straussler-Scheinker病一例报告
Zeran Chen,Junjun Guo,Ningjing Ran et al.
Zeran Chen et al.
Gerstmann-Sträussler-Scheinker (GSS) disease is an autosomal dominant neurodegenerative disease, and it is characterized by progressive cerebellar ataxia. Up to now, GSS cases with the p.P102L mutation have mainly been reported in Caucasia...
Case Reports
Prion. 2023 Dec;17(1):37-43. DOI:10.1080/19336896.2023.2180255 2023
Differential involvement of amyloidogenic evolvability in oligodendropathies; Multiple Sclerosis and Multiple System Atrophy [0.03%]
淀粉样蛋白致病性的不同作用：多发性硬化和多系统萎缩
Jianshe Wei,Gilbert Ho,Eliezer Masliah et al.
Jianshe Wei et al.
Although multiple sclerosis (MS) and multiple system atrophy (MSA) are both characterized by impaired oligodendrocytes (OLs), the aetiological relevance remains obscure. Given inherent stressors affecting OLs, the objective of the present s...
Inga Zerr
Inga Zerr
Canine detection of chronic wasting disease (CWD) in laboratory and field settings [0.03%]
实验室和野外环境中犬类探测慢性消耗性疾病的试验
Amritha Mallikarjun,Ben Swartz,Sarah A Kane et al.
Amritha Mallikarjun et al.
Chronic wasting disease (CWD) is a fatal transmissible spongiform encephalopathy that affects both free-ranging and farmed cervid species, including mule deer, white-tailed deer, and elk (Odocoileus hemionus, Odocoileus virginianus, and Cer...
Anthony Ness,Judd Aiken,Debbie McKenzie
Anthony Ness
Eighteenth-century England witnessed the emergence of two neurological diseases in animals. Scrapie, a transmissible spongiform encephalopathy, is a fatal neurodegenerative disease of sheep and goats that appears in classical and atypical f...
Copper coordination modulates prion conversion and infectivity in mammalian prion proteins [0.03%]
铜配合物的调节能改变哺乳动物朊病毒中的朊病毒转换和感染力
Giuseppe Legname
Giuseppe Legname
In mammals the cellular form of the prion protein (PrPC) is a ubiquitous protein involved in many relevant functions in the central nervous system. In addition to its physiological functions PrPC plays a central role in a group of invariabl...
Kyu Hwan Shim,Niti Sharma,Seong Soo A An
Kyu Hwan Shim
Prion diseases are a group of incurable zoonotic neurodegenerative diseases (NDDs) in humans and other animals caused by the prion proteins. The abnormal folding and aggregation of the soluble cellular prion proteins (PrPC) into scrapie iso...
Variability in prion protein genotypes by spatial unit to inform susceptibility to chronic wasting disease [0.03%]
根据空间单元中朊病毒蛋白基因型的变异性来确定慢性消耗性疾病的易感性
Alberto F Fameli,Jessie Edson,Jeremiah E Banfield et al.
Alberto F Fameli et al.
Chronic wasting disease (CWD) is a fatal encephalopathy affecting North American cervids. Certain alleles in a host's prion protein gene are responsible for reduced susceptibility to CWD. We assessed for the first time variability in the pr...
18F-FP-CIT PET/CT in a case of probable sporadic Creutzfeldt-Jakob disease with parkinsonism as initial symptom [0.03%]
一例以帕金森氏病表现为首发症状的可能散发性克雅病患者的18F-FP-CIT正电子发射断层扫描/计算机体层摄影检查结果报告
Songhan Tang,Xiaofeng Dou,Ying Zhang
Songhan Tang
Creutzfeldt-Jakob disease (CJD) is a low-prevalence, fatal neurodegenerative disease. Parkinsonism as first symptom of CJD is rare. We present a case manifesting difficulty falling asleep as unspecific prodromal symptom and parkinsonism as ...
Case Reports
Prion. 2022 Dec;16(1):91-94. DOI:10.1080/19336896.2022.2093078 2022