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Your search keyword '"Zhou, Miaojin"' showing total 9 results
Start Over Author "Zhou, Miaojin" Publication Year Range Last 50 years Journal international journal of molecular sciences
9 results on '"Zhou, Miaojin"'

4. Damaged DNA Is an Early Event of Neurodegeneration in Induced Pluripotent Stem Cell-Derived Motoneurons with UBQLN2P497H Mutation

Author

Zhang, Yiti, primary, Zeng, Baitao, additional, Gu, Ao, additional, Kang, Qinyu, additional, Zhao, Mingri, additional, Peng, Guangnan, additional, Zhou, Miaojin, additional, Liu, Wanxi, additional, Liu, Min, additional, Ding, Lingjie, additional, Liang, Desheng, additional, Liu, Xionghao, additional, and Liu, Mujun, additional

Published
2022
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8. Damaged DNA Is an Early Event of Neurodegeneration in Induced Pluripotent Stem Cell-Derived Motoneurons with UBQLN2 P497H Mutation.

Author

Zhang, Yiti, Zeng, Baitao, Gu, Ao, Kang, Qinyu, Zhao, Mingri, Peng, Guangnan, Zhou, Miaojin, Liu, Wanxi, Liu, Min, Ding, Lingjie, Liang, Desheng, Liu, Xionghao, and Liu, Mujun

Subjects
MOTOR neurons, AMYOTROPHIC lateral sclerosis, DNA damage, GENETIC mutation, FRONTOTEMPORAL dementia, DNA repair
Abstract

Ubiquilin-2 (UBQLN2) mutations lead to familial amyotrophic lateral sclerosis (FALS)/and frontotemporal dementia (FTLD) through unknown mechanisms. The combination of iPSC technology and CRISPR-mediated genome editing technology can generate an iPSC-derived motor neuron (iPSC-MN) model with disease-relevant mutations, which results in increased opportunities for disease mechanism research and drug screening. In this study, we introduced a UBQLN2-P497H mutation into a healthy control iPSC line using CRISPR/Cas9, and differentiated into MNs to study the pathology of UBQLN2-related ALS. Our in vitro MN model faithfully recapitulated specific aspects of the disease, including MN apoptosis. Under sodium arsenite (SA) treatment, we found differences in the number and the size of UBQLN2+ inclusions in UBQLN2P497H MNs and wild-type (WT) MNs. We also observed cytoplasmic TAR DNA-binding protein (TARDBP, also known as TDP-43) aggregates in UBQLN2P497H MNs, but not in WT MNs, as well as the recruitment of TDP-43 into stress granules (SGs) upon SA treatment. We noted that UBQLN2-P497H mutation induced MNs DNA damage, which is an early event in UBQLN2-ALS. Additionally, DNA damage led to an increase in compensation for FUS, whereas UBQLN2-P497H mutation impaired this function. Therefore, FUS may be involved in DNA damage repair signaling. [ABSTRACT FROM AUTHOR]

Published
2022
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