Single gene tweak for prion diseases

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Single gene tweak for prion diseases

机译：gene病毒疾病的单基因调整

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Engineered mouse strains develop rare prion diseases without the need for an infectious agent. Susan Lindquist of the Whitehead Institute for Biomedical Research in Cambridge, Massachusetts, and her colleagues have created mouse models for two fatal human diseases caused by prions-misfolded proteins that spur other proteins to misfold. Creutzfeldt-Jakob disease and fatal familial insomnia affect different parts of the brain and arise from different mutations in the same gene. Rather than inserting mutated genes into the mouse genome-a tactic that ineffectively mimics the disease - the researchers mutated the prion gene in place.

机译：经过改造的小鼠品系无需感染剂即可发展罕见的病毒疾病。马萨诸塞州剑桥市怀特黑德生物医学研究所的苏珊·林德奎斯特（Susan Lindquist）和她的同事们创建了两种由fold病毒错折叠的蛋白质引起的致命人类疾病的小鼠模型，这些蛋白质刺激其他蛋白质错折叠。克雅氏病和致命的家族性失眠症会影响大脑的不同部位，并且源于同一基因的不同突变。研究人员没有将突变的基因插入小鼠基因组中（一种无效地模仿这种疾病的策略），而是将the病毒基因进行了适当的突变。

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《Nature》 |2013年第7464期|505-505|共1页
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收录信息美国《科学引文索引》(SCI);美国《工程索引》(EI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
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1. A Single Amino Acid Substitution, Found in Mammals with Low Susceptibility to Prion Diseases, Delays Propagation of Two Prion Strains in Highly Susceptible Transgenic Mouse Models [J] . Otero Alicia, Hedman Carlos, Fernandez-Borges Natalia, Molecular Neurobiology . 2019,第9期

机译：在对朊病毒疾病的敏感性低的哺乳动物中发现单一的氨基酸取代，在高易感转基因小鼠模型中延迟了两种朊病毒菌株的繁殖
2. The unfolded state of the murine prion protein and properties of single-point mutants related to human prion diseases. [J] . Gerum C, Schlepckow K, Schwalbe H Journal of Molecular Biology . 2010,第1期

机译：鼠病毒蛋白的展开状态和与人类病毒疾病有关的单点突变体的特性。
3. We shall overcome prion diseases only by using both scientific and empiric findings or why honoring discovery and development of avermectins with 2015 Nobel Prize for physiology or medicine does not decrease their chronic toxicity especially neural degeneration increasing the susceptibility for prion diseases [J] . Becker Andreas Prion . 2016,第Suppla1期

机译：我们只能通过科学和经验性的发现来克服ion病毒疾病，或者为什么要凭借2015年诺贝尔生理学或医学奖纪念阿维菌素的发现和开发不会降低其慢性毒性，尤其是神经退行性疾病而增加对病毒疾病的敏感性
4. Role of Prion Protein Anchoring in Pathogenesis of Prion Diseases [C] . B. Chesebro Annual Meeting of the American Society for Veterinary Clinical Pathology . 2007

机译：朊蛋白锚定在朊病毒疾病发病机制中的作用
5. Characterizing the Demographic History and Prion Protein Gene Variation to Infer Susceptibility to Chronic Wasting Disease in a Na?ve Population of White-Tailed Deer (Odocoileus virginianus) [D] . Haworth, Sarah Elizabeth. 2021

机译：表征人口统计历史和朊病毒蛋白基因变异以推断对白尾鹿（Odocoileus virginianus）的Na've群中慢性浪费疾病的敏感性
6. Absence of Strong Genetic Linkage Disequilibrium between Single Nucleotide Polymorphisms (SNPs) in the Prion Protein Gene (PRNP) and the Prion-Like Protein Gene (PRND) in the Horse a Prion-Resistant Species [O] . Sae-Young Won, Yong-Chan Kim, Kyoungtag Do, 2020

机译：抵抗Pri病毒的马的on病毒蛋白基因（PRNP）和象Pri病毒蛋白基因（PRND）的单核苷酸多态性（SNP）之间不存在强的遗传连锁不平衡
7. A Single Amino Acid Substitution, Found in Mammals with Low Susceptibility to Prion Diseases, Delays Propagation of Two Prion Strains in Highly Susceptible Transgenic Mouse Models [O] . Alicia Otero, Carlos Hedman, Natalia Fernández-Borges, 2019

机译：在哺乳动物中发现的单一氨基酸取代，对朊病毒疾病的低敏感性，在高易感转基因小鼠模型中延迟了两种朊病毒菌株的繁殖

Single gene tweak for prion diseases

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