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An oligomer-specific antibody improved motor function and attenuated neuropathology in the SOD1-G93A transgenic mouse model of ALS
Dong, QX; Zhu, J; Liu, SY; Yu, XL; Liu, RT; Dong, Quan-xiu; Zhu, Jie; Liu, Shu-ying; Yu, Xiao-lin; Liu, Rui-tian
2018
Source PublicationINTERNATIONAL IMMUNOPHARMACOLOGY
ISSN1567-5769
Volume65Pages:413
Abstract

Amyotrophic lateral sclerosis (ALS) is a rapidly progressing neurodegenerative disease characterized by motor neuron loss in the brain and spinal cord. Mutations in Cu-Zn superoxide dismutase (SOD1) are the first identified genetic mutations that are causative for familial ALS. Soluble SOD1 oligomers are considered the most toxic species and play a key role in the pathologic process of ALS. Here we present a therapeutic strategy for ALS with an oligomer-specific antibody (W20) targeting toxic SOD1 oligomers. Our study showed that W20 significantly improved motor neuron survival and motor performance in SOD1-G93A mouse model of ALS when administrated even at low dose within short time. Further investigation demonstrated that the beneficial effects of W20 resulted from the reduction of SOD1 oligomer levels and the inhibition of gliosis and neuroinflammation in the spinal cords and brain stems of ALS model mice. These findings for the first time suggest that an oligomer-specific antibody has promising therapeutic potential for ALS and open a new way for ALS treatment.

KeywordAmyotrophic Lateral Sclerosis (Als) Amyotrophic-lateral-sclerosis Sod1 Molecular Pathways Motor Neuron Spinal-cord Oligomer Dismutase Single-chain Variable Fragment Antibody Neurodegeneration Aggregation Mechanism Recognize Genes Cells
SubtypeArticle
DOI10.1016/j.intimp.2018.10.032
WOS IDWOS:000452940500047
Citation statistics
Document Type期刊论文
Identifierhttp://ir.ipe.ac.cn/handle/122111/26744
Collection中国科学院过程工程研究所
Recommended Citation
GB/T 7714
Dong, QX,Zhu, J,Liu, SY,et al. An oligomer-specific antibody improved motor function and attenuated neuropathology in the SOD1-G93A transgenic mouse model of ALS[J]. INTERNATIONAL IMMUNOPHARMACOLOGY,2018,65:413.
APA Dong, QX.,Zhu, J.,Liu, SY.,Yu, XL.,Liu, RT.,...&Liu, Rui-tian.(2018).An oligomer-specific antibody improved motor function and attenuated neuropathology in the SOD1-G93A transgenic mouse model of ALS.INTERNATIONAL IMMUNOPHARMACOLOGY,65,413.
MLA Dong, QX,et al."An oligomer-specific antibody improved motor function and attenuated neuropathology in the SOD1-G93A transgenic mouse model of ALS".INTERNATIONAL IMMUNOPHARMACOLOGY 65(2018):413.
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