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

ATAXIN-2 intermediate-length polyglutamine expansions elicit ALS-associated metabolic and immune phenotypes

共济失调蛋白-2中等长度的多聚谷氨酰胺扩增可引发肌萎缩侧索硬化症相关的代谢和免疫表型

Renata Vieira de Sá # ,Emma Sudria-Lopez # ,Marta Cañizares Luna # ,Oliver Harschnitz ,Dianne M A van den Heuvel ,Sandra Kling ,Danielle Vonk ,Henk-Jan Westeneng ,Henk Karst ,Lauri Bloemenkamp ,Suzy Varderidou-Minasian ,Domino K Schlegel ,Mayte Mars ,Mark H Broekhoven ,Nicky C H van Kronenburg ,Youri Adolfs ,Vamshidhar R Vangoor ,Rianne de Jongh ,Tijana Ljubikj ,Lianne Peeters ,Sabine Seeler ,Enric Mocholi ,Onur Basak ,David Gordon ,Fabrizio Giuliani ,Tessa Verhoeff ,Giel Korsten ,Teresa Calafat Pla ,Morten T Venø ,Jørgen Kjems ,Kevin Talbot ,Michael A van Es ,Jan H Veldink ,Leonard H van den Berg ,Pavol Zelina ,R Jeroen Pasterkamp

Dynactin-1 mediates rescue of impaired axonal transport due to reduced mitochondrial bioenergetics in amyotrophic lateral sclerosis motor neurons

Dynactin-1 介导修复肌萎缩侧索硬化症运动神经元中因线粒体生物能量减少而受损的轴突运输

Ruxandra Dafinca, Carlota Tosat-Bitrian, Emily Carroll, Björn F Vahsen, Javier Gilbert-Jaramillo, Jakub Scaber, Emily Feneberg, Errin Johnson, Kevin Talbot

An ALS-associated mutation dysregulates microglia-derived extracellular microRNAs in a sex-specific manner

一种与肌萎缩侧索硬化症 (ALS) 相关的突变以性别特异性的方式失调小胶质细胞来源的细胞外微RNA。

Eleni Christoforidou ,Libby Moody ,Greig Joilin ,Fabio A Simoes ,David Gordon ,Kevin Talbot ,Majid Hafezparast

Mutant GGGGCC RNA prevents YY1 from binding to Fuzzy promoter which stimulates Wnt/β-catenin pathway in C9ALS/FTD

突变型 GGGGCC RNA 阻止 YY1 与 Fuzzy 启动子结合,从而抑制 C9ALS/FTD 中的 Wnt/β-catenin 通路。

Zhefan Stephen Chen ,Mingxi Ou ,Stephanie Taylor ,Ruxandra Dafinca ,Shaohong Isaac Peng ,Kevin Talbot ,Ho Yin Edwin Chan

PRMT inhibitor promotes SMN2 exon 7 inclusion and synergizes with nusinersen to rescue SMA mice

PRMT 抑制剂促进 SMN2 外显子 7 插入并与 nusinersen 协同作用挽救 SMA 小鼠

Anna J Kordala, Jessica Stoodley, Nina Ahlskog, Muhammad Hanifi, Antonio Garcia Guerra, Amarjit Bhomra, Wooi Fang Lim, Lyndsay M Murray, Kevin Talbot, Suzan M Hammond, Matthew Ja Wood, Carlo Rinaldi

Atypical TDP-43 protein expression in an ALS pedigree carrying a p.Y374X truncation mutation in TARDBP

在携带TARDBP基因p.Y374X截断突变的ALS家系中,TDP-43蛋白表达异常。

Johnathan Cooper-Knock ,Thomas H Julian ,Emily Feneberg ,J Robin Highley ,Maurice Sidra ,Martin R Turner ,Kevin Talbot ,Olaf Ansorge ,Scott P Allen ,Tobias Moll ,Tatyana Shelkovnikova ,Lydia Castelli ,Guillaume M Hautbergue ,Christopher Hewitt ,Janine Kirby ,Stephen B Wharton ,Richard J Mead ,Pamela J Shaw

Stress granule assembly in vivo is deficient in the CNS of mutant TDP-43 ALS mice

突变型 TDP-43 ALS 小鼠的中枢神经系统缺乏体内应激颗粒组装

Alicia Dubinski, Myriam Gagné, Sarah Peyrard, David Gordon, Kevin Talbot, Christine Vande Velde

Human stem cell models of neurodegeneration: From basic science of amyotrophic lateral sclerosis to clinical translation

神经退行性疾病的人类干细胞模型:从肌萎缩侧索硬化症的基础科学到临床转化

Elisa Giacomelli, Björn F Vahsen, Elizabeth L Calder, Yinyan Xu, Jakub Scaber, Elizabeth Gray, Ruxandra Dafinca, Kevin Talbot, Lorenz Studer

Poly(ADP-ribose) promotes toxicity of C9ORF72 arginine-rich dipeptide repeat proteins

聚(ADP-核糖)促进 C9ORF72 富含精氨酸的二肽重复蛋白的毒性

Junli Gao, Quinlan T Mewborne, Amandeep Girdhar, Udit Sheth, Alyssa N Coyne, Ritika Punathil, Bong Gu Kang, Morgan Dasovich, Austin Veire, Mariely DeJesus Hernandez, Shuaichen Liu, Zheng Shi, Ruxandra Dafinca, Elise Fouquerel, Kevin Talbot, Tae-In Kam, Yong-Jie Zhang, Dennis Dickson, Leonard Petruce

Human iPSC co-culture model to investigate the interaction between microglia and motor neurons

利用人诱导多能干细胞共培养模型研究小胶质细胞与运动神经元之间的相互作用

Björn F Vahsen ,Elizabeth Gray ,Ana Candalija ,Kaitlyn M L Cramb ,Jakub Scaber ,Ruxandra Dafinca ,Antigoni Katsikoudi ,Yinyan Xu ,Lucy Farrimond ,Richard Wade-Martins ,William S James ,Martin R Turner ,Sally A Cowley ,Kevin Talbot