SOD1

作品数：154被引量：340H指数：9
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相关作者：刘长林陈杰梁毅陈建雄李旭东更多>>
相关机构：华中师范大学河北医科大学河北医科大学第二医院武汉大学更多>>
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GCN2 inhibition reduces mutant SOD1 clustering and toxicity and delays disease progression in an amyotrophic lateral sclerosis mouse model
《Translational Neurodegeneration》2024年第1期351-355,共5页Didio Alberto Ortiz Nuria Peregrín Miguel Valencia Rodrigo Vinueza-Gavilanes Elisa Marín-Ordovas Roberto Ferrero María Jesús Nicolás Gloria González-Aseguinolaza Montserrat Arrasate Tomás Aragón 
supported by PID2020-120497RB-I00 MCIU/AEI/https://doi.org/10.13039/501100011033,BFU2017-90043-P MCINN/AEI/https://doi.org/10.13039/501100011033/;by FEDER“Una manera de hacer Europa”(MA and TA),Proyecto Intramural IdisNa 2022(MA),Fundación para la Investigación Médica Aplicada(FIMA)Proyectos I+D,2017(TA)and Fundación Occident and DalecandELA Association(MA);supported by República de Panamá,Programa de Becas IFARHU-SENACYT(reference number 270-2018-922),NP by AC FIMA pre-doctoral fellowship.
Main text The disruption of protein folding homeostasis in motoneurons(MNs)and the accumulation of protein aggregates are some of the main molecular hallmarks of amyotrophic lateral sclerosis(ALS).Evidence from sporad...
关键词：HOMEOSTASIS amyotrophic SCLEROSIS 
Variability in SOD1-associated amyotrophic lateral sclerosis:geographic patterns,clinical heterogeneity,molecular alterations,and therapeutic implications
《Translational Neurodegeneration》2024年第1期717-735,共19页Miaodan Huang Yong ULiu Xiaoli Yao Dajiang Qin Huanxing Su 
supported by Macao Science and Technology Development Fund(0061/2021/A2);Shenzhen Science and Technology Innovation Commission(EF026/ICMS-SHX/2022/SZSTIC).
Amyotrophic lateral sclerosis(ALS)is a fatal neurodegenerative disease characterized by progressive loss of motor neurons,resulting in global health burden and limited post-diagnosis life expectancy.Although primarily...
关键词：Amyotrophic lateral sclerosis SOD1 variants GENOTYPE Geographic pattern Genetic therapy 
Autologous treatment for ALS with implication for broad neuroprotection
《Translational Neurodegeneration》2022年第1期718-736,共19页Daehwan Kim Subin Kim Ashley Sung Neetika Patel Nathan Wong Michael J.Conboy Irina M.Conboy 
NIBIB(R01 EB023776),NIA(R01AG071787),NHLBI(R01 HL139605),Open Philanthropy,Silicon Valley Community Foundation,Foster Foundation,San Francisco Foundation,Georges’Harik Foundation,and Donors’Trust grants to IC.
Background: Amyotrophic lateral sclerosis (ALS) is characterized by a progressive loss of motor neurons (MNs), leading to paralysis, respiratory failure and death within 2-5 years of diagnosis. The exact mechanisms of...
关键词：NEURODEGENERATION Amyotrophic lateral sclerosis SOD1 mutation Pluripotent stem cells SECRETOME 
A natural history comparison Check for updates of SOD1-mutant patients with amyotrophic lateral sclerosis between Chinese and German populations被引量：1
《Translational Neurodegeneration》2021年第4期551-553,共3页Lu Tang Johannes Dorst Lu Chen Xiaolu Liu Yan Ma Komelia Günther Sebastian Michels Kathrin Müller Axel Freischmidt Jochen H.Weishaupt Dongsheng Fan Albert C.Ludolph 
supported by grants from the National Natural Science Foundation of China(81873784,82071426 and 81901298);Clinical Cohort Construction Program of Peking University Third Hospital(BYSYDL2019002).
Currently,there is no effective treatment for amyotrophic lateral sclerosis(ALS),despite the limited efficacy of riluzole[1]and edaravone[2].SOD1(coding for the Cu/Zn superoxide dismutase)is the second most frequent g...
关键词：SOD1 PATIENTS amyotrophic 
Downregulated miR-18b-5p triggers apoptosis by inhibition of calcium signaling and neuronal cell differentiation in transgenic SOD1(G93A)mice and SOD1(G17S and G86S)ALS patients被引量：2
《Translational Neurodegeneration》2020年第3期323-343,共21页Ki Yoon Kim Yu Ri Kim Kyung Won Choi Mijung Lee Somyung Lee Wooseok Im Je-Young Shin Jin Young Kim Yoon Ho Hong Manho Kim Jong-ll Kim Jung-Joon Sung 
This research was supported by the Brain Research Program through the National Research Foundation of Korea(NRF)funded by the Ministry of Science and ICT(2017M3C7A102536521 and 2018R1A5A202596413).
Background:MicroRNAs(miRNAs)are endogenous non-coding RNAS that regulate gene expression at the post-transcriptional level and are key modulators in neurodegenerative diseases.Overexpressed miRNAs play an important ro...
关键词：MIRNAS Hif1a Mef2c Mctp1 Rarb 
Better survival in female SOD1-mutant patients with ALS:a study of SOD1-related natural history被引量：1
《Translational Neurodegeneration》2019年第1期14-23,共10页Lu Tang Yan Ma Xiao-lu Liu Lu Chen Dong-sheng Fan 
This work was supported by the National Natural Science Foundation of China under Grant 81030019;Peking University-Ulm University Union Foundation under Grant PKU2017ZC001-2;the Natural Science Foundation of Beijing Municipality under Grant 7102161;the National Clinical Key Program of China.
Background:SOD1 mutations are the most common cause of amyotrophic lateral sclerosis(ALS)in non-Caucasian patients.Detailed natural history profiles of SOD1-mutant patients will be beneficial for the strategy and inte...
关键词：Amyotrophic lateral SCLEROSIS Natural history studies SOD1 MUTATIONS GENDER difference 
Correction to:Better survival in female SOD1-mutant patients with ALS: a study of SOD1-related natural history被引量：2
《Translational Neurodegeneration》2019年第1期126-126,共1页Lu Tang Yan Ma Xiao-lu Liu Lu Chen Dong-sheng Fan 
In the original publication of this article[1],a numerical value in the sentence“The mean(SD)AAO was 43.92 years(9.24)for all subjects,with a significant difference between patients carrying mutations in exon 2(n=24,...
关键词：SOD1 PATIENTS FEMALE 
Ultra-High Field Diffusion MRI Reveals Early Axonal Pathology in Spinal Cord of ALS mice被引量：3
《Translational Neurodegeneration》2018年第1期187-200,共14页Rodolfo G.Gatto Manish Y.Amin Daniel Deyoung Matthew Hey Thomas H.Mareci Richard L.Magin 
This study was supported in part by a Chicago Biomedical Consortium(CBC)postdoctoral fellowship grant(Award#085740)to RG at the University of Illinois in Chicago.
Background:Amyotrophic lateral sclerosis(ALS)is a disease characterized by a progressive degeneration of motor neurons leading to paralysis.Our previous MRI diffusion tensor imaging studies detected early white matter...
关键词：Amyotrophic Lateral Sclerosis Spinal Cord Ultra-high Field MRI Diffusion Tensor Imaging Yellow Fluorescent Protein G93A-SOD1 mice Axonal Degeneration TRACTOGRAPHY Connectomics 
Dynamic changes of CX3CL1/CX3CR1 axis during microglial activation and motor neuron loss in the spinal cord of ALS mouse model被引量：10
《Translational Neurodegeneration》2018年第1期378-391,共14页Jingjing Zhang Yufei Liu Xinyao Liu Song Li Cheng Cheng Sheng Chen Weidong Le 
This work was supported by funding from the National Natural Sciences Foundation of China(NSFC 81430021 and 81370470);the Program for Liaoning Innovative Research Team in University(LT2015009);Liaoning Science and Technology Project(2015225008).
Background:Neuron-microglia communication plays a crucial role in the motor neurons(MNs)death in amyotrophic lateral sclerosis(ALS).Neurons can express chemokine(C-X3-C motif)ligand 1(CX3CL1),which mediates microglial...
关键词：ALS CX3CL1/CX3CR1 AXIS MICROGLIAL activation SOD1^(G93A) mice 
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