日期:
2020 年 — 2026 年
2020
2021
2022
2023
2024
2025
2026
影响因子:

Advancing prion diagnostics: full-length human E200K RT-QuIC substrate facilitates prion detection in tear fluid and improves sensitivity in cerebrospinal fluid

推进朊病毒诊断:全长人E200K RT-QuIC底物有助于检测泪液中的朊病毒并提高脑脊液中的检测灵敏度

Correia, Susana Da Silva; Schmitz, Matthias; Hermann, Peter; Goebel, Stefan; Gerecke, Jaqueline; Lingor, Paul; Maass, Fabian; Fischer, Anna-Lisa; Canaslan, Sezgi; Eraña, Hasier; Castilla, Joaquín; Da Silva Correia, Angela; Zerr, Inga

Overexpression of bank vole PrP(I109) in mice induces a spontaneous atypical prion disease with sex-dependent onset, early NfL elevation, and universal prion strain permissiveness

小鼠体内田鼠 PrP(I109) 的过表达可诱发一种自发性非典型朊病毒病,该病具有性别依赖性发病、早期 NfL 水平升高以及对普遍朊病毒株的易感性。

Eraña, Hasier; Vidal, Enric; Fernández-Borges, Natalia; Charco, Jorge M; Díaz-Domínguez, Carlos M; Sampedro-Torres-Quevedo, Cristina; San-Juan-Ansoleaga, Maitena; Fernández-Muñoz, Eva; Galarza-Ahumada, Josu; Pérez-Castro, Miguel Ángel; Gonçalves-Anjo, Nuno; Piñeiro, Patricia; Pirisinu, Laura; Di Bari, Michele Angelo; Giler, Samanta; Raimondi, Ilaria; Espinosa, Juan Carlos; Vanni, Ilaria; D'Agostino, Claudia; Rodríguez-Cuesta, Juan; Pasetto, Laura; Bonetto, Valentina; González-Martín, Nora; Teijeira, Susana; Zou, Wen-Quan; Geijo, Mariví; Torres, Juan María; Chiesa, Roberto; Sánchez-Martín, Manuel A; Nonno, Romolo; Requena, Jesús R; Castilla, Joaquín

Transmission Ratio Distortion in Genetic Prion Diseases: Clarifying Methodological Considerations

遗传性朊病毒疾病的传播比率偏差:方法学考量澄清

Kortazar-Zubizarreta, Izaro; Manero-Azua, Africa; Eraña, Hasier; Castilla, Joaquín; Perez de Nanclares, Guiomar

Variably Protease-Sensitive Prionopathy: Two New Cases With Motor Neuron-Dementia Syndrome

变异型蛋白酶敏感性朊病毒病:两例伴有运动神经元痴呆综合征的新病例

Erro, María Elena; Zelaya, María Victoria; Eraña, Hasier; de Gordoa, Javier Sánchez Ruiz; García-Amigot, Fermín; Simonovska-Serra, Anika; Caballero, María Cristina; Ferrer, Isidre; Gelpi, Ellen; Jericó, Ivonne; Castilla, Joaquín

Leveraging the dominant-negative effect of the kuru-protective G127V prion protein variant as a novel therapeutic strategy

利用库鲁病保护性朊病毒蛋白变体G127V的显性负效应作为一种新型治疗策略

Gatdula, Jean R P; Orbe, Isabel C; Tolton, Samantha G; Saunders, Linnea M; Vultaggio, Janelle S; Mercer, Robert C C; Bartz, Jason C; Telling, Glenn C; Eraña, Hasier; Castilla, Joaquín; Harris, David A

Characterization of prion strains and peripheral prion infectivity patterns in E200K genetic CJD patients

E200K基因型克雅氏病患者朊病毒株和外周朊病毒感染模式的特征分析

Barrio, Tomás; Douet, Jean-Yves; Žáková, Dana; Eraña, Hasier; Huor, Alvina; Cassard, Hervé; Alzuguren, Oihane; Lugan, Séverine; Aron, Naïma; Péran, Patrice; Castilla, Joaquín; Andréoletti, Olivier

Spontaneous generation of diverse recombinant prion strains: sulfated glycan cofactors facilitate strain emergence but do not determine specific strain properties

自发产生多种重组朊病毒株:硫酸化聚糖辅因子促进毒株出现,但不决定毒株的具体特性

Lorenzo, Nuria L; Eraña, Hasier; Vidal, Enric; Charco, Jorge M; Parga, Lucía; Díaz-Domínguez, Carlos M; Veiga, Sonia; Bravo, Susana; Giler, Samanta; Geijo, Mariví; Requena, Jesús R; Castilla, Joaquín

Conservation of strain properties of bank vole-adapted chronic wasting disease in the absence of glycosylation and membrane anchoring

在缺乏糖基化和膜锚定的情况下,田鼠适应性慢性消耗性疾病的菌株特性得以保留

Vidal, Enric; Eraña, Hasier; Charco, Jorge M; Lorenzo, Nuria L; Giler, Samanta; Ordóñez, Montserrat; Fernández-Muñoz, Eva; San-Juan-Ansoleaga, Maitena; Telling, Glenn C; Sánchez-Martín, Manuel A; Geijo, Mariví; Requena, Jesús R; Castilla, Joaquín

Effect of primary structural variation on cervid prion protein in flexibility, stability, and spontaneous misfolding propensity

一级结构变异对鹿朊病毒蛋白的柔性、稳定性和自发错误折叠倾向的影响

Díaz-Domínguez, Carlos M; Eraña, Hasier; Peccati, Francesca; Vidal, Enric; Charco, Jorge M; Sampedro-Torres-Quevedo, Cristina; Pérez-Castro, Miguel A; Lorenzo, Nuria L; Giler, Samanta; Telling, Glenn C; Geijo, Mariví; Requena, Jesús R; Jiménez-Osés, Gonzalo; Castilla, Joaquín

A comprehensive phylogeny of mammalian PRNP gene reveals no influence of prion misfolding propensity on the evolution of this gene

对哺乳动物PRNP基因的全面系统发育分析表明,朊病毒错误折叠倾向对该基因的进化没有影响。

Sampedro-Torres-Quevedo, Cristina; Eraña, Hasier; Charco, Jorge M; Díaz-Domínguez, Carlos M; San-Juan-Ansoleaga, Maitena; Fernández-Muñoz, Eva; Gonçalves-Anjo, Nuno; Galarza-Ahumada, Josu; Cortazar, Ana R; Nespolo, Roberto F; Quintero-Galvis, Julian F; Manero-Azua, Africa; Polanco-Alonso, Diego; Gaite-Reguero, Adrián; Olalde, Íñigo; Marigorta, Urko M; Perez de Nanclares, Guiomar; Aransay, Ana M; Castilla, Joaquín