The first Chinese case of Creutzfeldt-Jakob disease patient with R208H mutation in PRNP [0.03%]
中国首例PRNP基因R208H突变的克雅病患者
Cao Chen,Qi Shi,Chan Tian et al.
Cao Chen et al.
A case of Creutzfeldt-Jakob disease (CJD) with a rare mutation of the prion protein (PrP) gene (PRNP) at codon 208 (R208H), while the codon 129 was a methionine homozygous genotype is reported. The patient initial displayed hand tremor, diz...
PrP assemblies: spotting the responsible regions in prion propagation [0.03%]
朊蛋白结构装配：在朊病毒传播过程中识别负责区域
Stéphanie Prigent,Human Rezaei
Stéphanie Prigent
The "protein only" hypothesis states that the key phenomenon in prion pathogenesis is the conversion of the host protein (PrPC) into a b-sheet enriched polymeric and pathogenic conformer (PrPSc). However the region of PrP bearing the inform...
Real-time quaking-induced conversion: a highly sensitive assay for prion detection [0.03%]
实时震颤诱导转化：一种用于朊病毒检测的高灵敏度测定法
Ryuichiro Atarashi,Kazunori Sano,Katsuya Satoh et al.
Ryuichiro Atarashi et al.
We recently developed a new in vitro amplification technology, designated "real-time quaking-induced conversion (RT-QUIC)", for detection of the abnormal form of prion protein (PrPSc) in easily accessible specimens such as cerebrospinal flu...
Aerosols: an underestimated vehicle for transmission of prion diseases? [0.03%]
气溶胶：一种被低估的传播朊病毒疾病的方式？
Lothar Stitz,Adriano Aguzzi
Lothar Stitz
We and others have recently reported that prions can be transmitted to mice via aerosols. These reports spurred a lively public discussion on the possible public-health threats represented by prion-containing aerosols. Here we offer our vie...
Allelic frequency and genotypes of prion protein at codon 136 and 171 in Iranian Ghezel sheep breeds [0.03%]
伊朗格赫泽尔绵羊品种第136和171密码子的朊病毒蛋白等位基因频率及基因型
Siamak Salami,Reza Ashrafi Zadeh,Mir Davood Omrani et al.
Siamak Salami et al.
PrP genotypes at codons 136 and 171 in one hundred twenty Iranian Ghezel sheep breeds were studied using allele-specific PCR amplification and compared with the well-known sheep breeds in North America, the United States, and Europe. The fr...
The cellular prion protein with a monoacylated glycosylphosphatidylinositol anchor modifies cell membranes, inhibits cell signaling and reduces prion formation [0.03%]
单酰化糖基磷脂酰肌醇锚定的细胞朊病毒蛋白改变细胞膜、抑制细胞信号传导并减少朊病毒形成
Clive Bate,Alun Williams
Clive Bate
The prion diseases occur following the conversion of the cellular prion protein (PrPC) into a disease-related isoform (PrPSc). In this study a cell painting technique was used to examine the role of the glycosylphosphatidylinositol (GPI) an...
Insights into prion biology: integrating a protein misfolding pathway with its cellular environment [0.03%]
朊病毒生物学新见解：整合蛋白质错误折叠途径与细胞内环境的新模式
Susanne DiSalvo,Tricia R Serio
Susanne DiSalvo
Protein misfolding and assembly into ordered, self-templating aggregates (amyloid) has emerged as a novel mechanism for regulating protein function. For a subclass of amyloidogenic proteins known as prions, this process induces transmissibl...
Eri Saijo,Stephen W Scheff,Glenn C Telling
Eri Saijo
The suggested role of cellular prion protein (PrP (C) ) in mediating the toxic effects of oligomeric amyloid β peptide (Aβ) in Alzheimer disease (AD) is controversial. To address the hypothesis that variable PrP (C) expression is involved...
Photodegradation illuminates the role of polyanions in prion infectivity [0.03%]
光降解阐明了多阴离子在朊病毒感染性中的作用
Justin R Piro,Surachai Supattapone
Justin R Piro
Understanding the mechanism by which prion infectivity is encoded by the misfolded protein PrP (Sc ) remains a high priority within the prion field. Work from several groups has indicated cellular cofactors may be necessary to form infectio...
Prion protein function and the disturbance of early embryonic development in zebrafish [0.03%]
朊蛋白功能及其对斑马鱼早期胚胎发育的影响
Mohasina Syed,Rasoul Nourizadeh-Lillabadi,Charles McL Press et al.
Mohasina Syed et al.
Transmissible Spongiform Encephalopathies (TSE) or prion diseases are a threat to food safety and to human and animal health. The molecular mechanisms responsible for prion diseases share similarities with a wider group of neurodegenerative...