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大鼠中敲除SOD1导致肌萎缩侧索硬化症(ALS)表型

LI Xinyue PAN Shuo ZHANG Lei HE Jiayue LIU Ning ZHANG Xu GAO Shan QI Xiaolong MA Yuanwu

中国实验动物学报2025，Vol.33Issue(11)：1598-1609,12.

中国实验动物学报2025，Vol.33Issue(11)：1598-1609,12.DOI:10.3969/j.issn.1005-4847.2025.11.004

大鼠中敲除SOD1导致肌萎缩侧索硬化症(ALS)表型

SOD1 knockout in rats leads to amyotrophic lateral sclerosis(ALS)

LI Xinyue ¹PAN Shuo ¹ZHANG Lei ¹HE Jiayue ¹LIU Ning ¹ZHANG Xu ¹GAO Shan ¹QI Xiaolong ¹MA Yuanwu²

作者信息

1. State Key Laboratory of Respiratory Health and Multimorbidity,NHC Key Laboratory of Human Disease Comparative Medicine,Key Laboratory of Pathogen Infection Prevention and Control Ministry of Education,Institute of Laboratory Animal Science,Chinese Academy of Medical Sciences&Comparative Medicine Center of Peking Union Medical College,Beijing 100021,China
2. State Key Laboratory of Respiratory Health and Multimorbidity,NHC Key Laboratory of Human Disease Comparative Medicine,Key Laboratory of Pathogen Infection Prevention and Control Ministry of Education,Institute of Laboratory Animal Science,Chinese Academy of Medical Sciences&Comparative Medicine Center of Peking Union Medical College,Beijing 100021,China||Medical Primate Research Center,Institute of Laboratory Animal Science,Chinese Academy of Medical Sciences,Beijing 100021,China||Haihe Laboratory of Cell Ecosystem,Tianjin 300301,China
折叠

摘要

Abstract

Objective To establish superoxide dismutase 1(SOD1)-knockout rats and analyze the neurological disease phenotypes.Methods SOD1-knockout rats were generated using CRISPR/Cas9 technology.Genotyping was performed using polymerase chain reaction and Sanger sequencing.Protein expression was validated by Western Blot.Knockout rats were crossed with wild-type rats to assess stable inheritance through generations.Phenotypic analyses included monitoring body mass and survival curves,histopathological examination,behavioral tests,motor neuron changes,and neuroinflammation.Results Genotypic analysis demonstrated successful establishment of SOD1-knockout rats,whichs showed altered morphology and reduced body mass,indicating impaired development.Behavioral tests also revealed impaired motor function,exhibiting amyotrophic lateral sclerosis(ALS)-like phenotypes.Pathological findings included reduced L4 motor neurons in the lumbar spinal cord,microglial infiltration,and decreased cell number and cross-sectional area in the gastrocnemius muscle.Conclusions We successfully established SOD1-knockout rats exhibiting key pathological features of ALS.This model provides a valuable platform for investigating the role of SOD1 role in the pathogenesis and mechanism of ALS.

关键词

SOD1/敲除型大鼠/肌萎缩侧索硬化症/ALS

Key words

superoxide dismutase 1/knockout rats/amyotrophic lateral sclerosis/ALS

分类

生物科学

引用本文复制引用

LI Xinyue,PAN Shuo,ZHANG Lei,HE Jiayue,LIU Ning,ZHANG Xu,GAO Shan,QI Xiaolong,MA Yuanwu..大鼠中敲除SOD1导致肌萎缩侧索硬化症(ALS)表型[J].中国实验动物学报,2025,33(11):1598-1609,12.

基金项目

中国医学科学院医学与健康科技创新工程(2021-I2M-1-024,2023-I2M-2-001),全国重点实验室专项经费(2060204),中央高校基本科研业务费(3332022040,3332023164),中国医学科学院公益性基本科研业务费(2023-PT180-01),细胞生态海河实验室创新基金(HH24KYZX0007). Funded by the CAMS Innovation Fund for Medical Sciences(CIFMS)(2021-I2M-1-024,2023-I2M-2-001),the Special Fund of the State Key Laboratory(2060204),the Fundamental Research Funds for the Central Universities(3332022040,3332023164),the Non-Profit Central Research Institute Fund of the Chinese Academy of Medical Sciences(2023-PT180-01),Haihe Laboratory of Cell Ecosystem Innovation Fund(HH24KYZX0007). （2021-I2M-1-024,2023-I2M-2-001）

中国实验动物学报

OA北大核心

ISSN：1005-4847

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