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Coactivation of GSK3β and IGF-1 Attenuates Amyotrophic Lateral Sclerosis Nerve Fiber Cytopathies in SOD1 Mutant Patient-Derived Motor Neurons.

Cells - 16 Oct 2021

Ting Hsiao-Chien, Yang Hui-I, Harn Horng-Jyh, Chiu Ing-Ming, Su Hong-Lin, Li Xiang, Chen Mei-Fang, Ho Tsung-Jung, Liu Ching-Ann, Tsai Yung-Jen, Chiou Tzyy-Wen, Lin Shinn-Zong, Chang Chia-Yu

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) is a progressive nervous system disease that causes motor neuron (MN) degeneration and results in patient death within a few years. To recapitulate the cytopathies of ALS patients' MNs, SOD1G85R mutant and corrected SOD1G85G isogenic-induced pluripotent stem cell (iPSC) lines were established. Two SOD1 mutant ALS (SOD1G85R and SOD1D90A), two SOD1 mutant corrected (SOD1G85G and...

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PMID: 34685754
DOI: 10.3390/cells10102773
PMCID: PMC8535155

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