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

A bacterial artificial chromosome mouse model of amyotrophic lateral sclerosis manifests 'space cadet syndrome' on two FVB backgrounds

肌萎缩侧索硬化症的细菌人工染色体小鼠模型在两种 FVB 背景下表现出“太空学员综合征”

Sophie E Badger, Ian Coldicott, Ergita Kyrgiou-Balli, Adrian Higginbottom, Chloé Moutin, Kamallia Mohd Imran, John C Day, Johnathan Cooper-Knock, Richard J Mead, James J P Alix

Pre-clinical development of AP4B1 gene replacement therapy for hereditary spastic paraplegia type 47

遗传性痉挛性截瘫47型AP4B1基因替代疗法的临床前开发

Jessica P Wiseman, Joseph M Scarrott, João Alves-Cruzeiro, Afshin Saffari, Cedric Böger, Evangelia Karyka, Emily Dawes, Alexandra K Davies, Paolo M Marchi, Emily Graves, Fiona Fernandes, Zih-Liang Yang, Ian Coldicott, Jennifer Hirst, Christopher P Webster, J Robin Highley, Neil Hackett, Adrienn Angy

Ap4b1-knockout mouse model of hereditary spastic paraplegia type 47 displays motor dysfunction, aberrant brain morphology and ATG9A mislocalization

遗传性痉挛性截瘫 47 型 Ap4b1 基因敲除小鼠模型表现出运动功能障碍、脑形态异常和 ATG9A 错误定位

Joseph M Scarrott, João Alves-Cruzeiro, Paolo M Marchi, Christopher P Webster, Zih-Liang Yang, Evangelia Karyka, Raffaele Marroccella, Ian Coldicott, Hannah Thomas, Mimoun Azzouz

An interaction between synapsin and C9orf72 regulates excitatory synapses and is impaired in ALS/FTD

突触蛋白与 C9orf72 之间的相互作用可调节兴奋性突触,并且在 ALS/FTD 中受损

Claudia S Bauer #, Rebecca N Cohen #, Francesca Sironi #, Matthew R Livesey, Thomas H Gillingwater, J Robin Highley, Daniel J Fillingham, Ian Coldicott, Emma F Smith, Yolanda B Gibson, Christopher P Webster, Andrew J Grierson, Caterina Bendotti, Kurt J De Vos

SPG15 protein deficits are at the crossroads between lysosomal abnormalities, altered lipid metabolism and synaptic dysfunction

SPG15 蛋白缺陷是溶酶体异常、脂质代谢改变和突触功能障碍的交汇点

Lara Marrone, Paolo M Marchi, Christopher P Webster, Raffaele Marroccella, Ian Coldicott, Steven Reynolds, João Alves-Cruzeiro, Zih-Liang Yang, Adrian Higginbottom, Mukhran Khundadze, Pamela J Shaw, Christian A Hübner, Matthew R Livesey, Mimoun Azzouz

C9orf72 expansion within astrocytes reduces metabolic flexibility in amyotrophic lateral sclerosis

星形胶质细胞内的 C9orf72 扩增降低了肌萎缩侧索硬化症的代谢灵活性

Scott P Allen, Benjamin Hall, Ryan Woof, Laura Francis, Noemi Gatto, Allan C Shaw, Monika Myszczynska, Jordan Hemingway, Ian Coldicott, Amelia Willcock, Lucy Job, Rachel M Hughes, Camilla Boschian, Nadhim Bayatti, Paul R Heath, Oliver Bandmann, Heather Mortiboys, Laura Ferraiuolo, Pamela J Shaw

Plastin 3 Promotes Motor Neuron Axonal Growth and Extends Survival in a Mouse Model of Spinal Muscular Atrophy

Plastin 3 促进运动神经元轴突生长并延长脊髓性肌萎缩症小鼠模型的生存期

Aziza Alrafiah, Evangelia Karyka, Ian Coldicott, Kayleigh Iremonger, Katherin E Lewis, Ke Ning, Mimoun Azzouz

C9orf72 expansion disrupts ATM-mediated chromosomal break repair

C9orf72 扩增破坏 ATM 介导的染色体断裂修复

Callum Walker, Saul Herranz-Martin, Evangelia Karyka, Chunyan Liao, Katherine Lewis, Waheba Elsayed, Vera Lukashchuk, Shih-Chieh Chiang, Swagat Ray, Padraig J Mulcahy, Mateusz Jurga, Ioannis Tsagakis, Tommaso Iannitti, Jayanth Chandran, Ian Coldicott, Kurt J De Vos, Mohamed K Hassan, Adrian Higginbo

Viral delivery of C9orf72 hexanucleotide repeat expansions in mice leads to repeat-length-dependent neuropathology and behavioural deficits

病毒在小鼠中传播 C9orf72 六核苷酸重复扩增会导致重复长度依赖性的神经病理学和行为缺陷

Saul Herranz-Martin, Jayanth Chandran, Katherine Lewis, Padraig Mulcahy, Adrian Higginbottom, Callum Walker, Isabel Martinez-Pena Y Valenzuela, Ross A Jones, Ian Coldicott, Tommaso Iannitti, Mohammed Akaaboune, Sherif F El-Khamisy, Thomas H Gillingwater, Pamela J Shaw, Mimoun Azzouz

Site Specific Modification of Adeno-Associated Virus Enables Both Fluorescent Imaging of Viral Particles and Characterization of the Capsid Interactome

腺相关病毒的位点特异性修饰可实现病毒颗粒的荧光成像和衣壳相互作用组的表征

Jayanth S Chandran, Paul S Sharp, Evangelia Karyka, João Miguel da Conceição Aves-Cruzeiro, Ian Coldicott, Lydia Castelli, Guillaume Hautbergue, Mark O Collins, Mimoun Azzouz