Abstract
Amyotrophic Lateral Sclerosis (ALS) is one of the commonest neurodegenerative diseases of adult-onset, which is characterized by the progressive death of motor neurons in the cerebral cortex, brain stem and spinal cord. The dysfunction and death of motor neurons lead to the progressive muscle weakness, atrophy, fasciculations, spasticity and ultimately the whole paralysis of body. Despite the identification of several genetic mutations associated with the pathogenesis of ALS, including mutations in chromosome 9 open reading frame 72 leading to the abnormal expansion of GGGGCC repeat sequence, TAR DNA-binding protein 43, fused in sarcoma/translocated in liposarcoma, copper/zinc superoxide dismutase 1 (SOD1) and TANK-binding kinase 1, the exact mechanisms underlying the specific degeneration of motor neurons that causes ALS remain incompletely understood. At present, since the transgenic model expressed SOD1 mutants was established, multiple in vitro models of ALS have been developed for studying the pathology, pathophysiology and pathogenesis of ALS as well as searching the effective neurotherapeutics. This review reviewed the details of present established in vitro models used in studying the pathology, pathophysiology and pathogenesis of ALS. Meanwhile, we also discussed the advantages, disadvantages, cost and availability of each models.
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References
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Acknowledgements
This review partially adapted and modified from the P81-84, 102-124 in the chapter 4 of book of In Vivo and In Vitro Models to Study Amyotrophic Lateral Sclerosis edited by François Berthod and François Gros-Louis in Centre LOEX de l'Université Laval, Centre de recherche FRSQ du Centre hospitalier affilié universitaire de Québec, Département de Chirurgie, Faculté de Médecine, Université Laval, Québec, Canada. We are honestly and sincerely grateful to them for their wonderful work here, and we also cited their book in our manuscript. We thank the National Natural Science Foundation of China (30560042, 81160161, 81360198 and 82160255), Education Department of Jiangxi Province (GJJ13198 and GJJ170021), Jiangxi Provincial Department of Science and Technology (20192BAB205043) and Health and Family Planning Commission of Jiangxi Province (20181019 and 202210002) for supporting this study.
Funding
The work was supported financially by the National Natural Science Foundation of China (30560042, 81160161, 81360198 and 82160255), Education Department of Jiangxi Province (GJJ13198 and GJJ170021), Jiangxi Provincial Department of Science and Technology (20192BAB205043) and Health and Family Planning Commission of Jiangxi Province (20181019 and 202210002) for supporting this study.
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LZ, WC and SJ conceptualized the manuscript, wrote, edited and reviewed the manuscript. LZ, and WC prepared the figures, wrote. LZ, RX corrected, amended and approved the finally edition of the manuscript.
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Zhou, L., Chen, W., Jiang, S. et al. In Vitro Models of Amyotrophic Lateral Sclerosis. Cell Mol Neurobiol 43, 3783–3799 (2023). https://doi.org/10.1007/s10571-023-01423-8
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DOI: https://doi.org/10.1007/s10571-023-01423-8