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Journal of Zhejiang University SCIENCE B 2019 Vol.20 No.5 P.449-456


Cell models and drug discovery for mitochondrial diseases

Author(s):  Shuang-Yi Hu, Qian-Qian Zhuang, Yue Qiu, Xu-Fen Zhu, Qing-Feng Yan

Affiliation(s):  Institute of Genetics and Regenerative Biology, College of Life Sciences, Zhejiang University, Hangzhou 310058, China; more

Corresponding email(s):   qfyan@zju.edu.cn, xufenzhu@zju.edu.cn

Key Words:  Mitochondrial diseases, Mitochondrial DNA, Cell model, Drug discovery

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Shuang-Yi Hu, Qian-Qian Zhuang, Yue Qiu, Xu-Fen Zhu, Qing-Feng Yan. Cell models and drug discovery for mitochondrial diseases[J]. Journal of Zhejiang University Science B, 2019, 20(5): 449-456.

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T1 - Cell models and drug discovery for mitochondrial diseases
A1 - Shuang-Yi Hu
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A1 - Xu-Fen Zhu
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J0 - Journal of Zhejiang University Science B
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PB - Zhejiang University Press & Springer
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DOI - 10.1631/jzus.B1900196

Mitochondrion is a semi-autonomous organelle, important for cell energy metabolism, apoptosis, the production of reactive oxygen species (ROS), and Ca2+ homeostasis. mitochondrial DNA (mtDNA) mutation is one of the primary factors in mitochondrial disorders. Though much progress has been made, there remain many difficulties in constructing cell models for mitochondrial diseases. This seriously restricts studies related to targeted drug discovery and the mechanism and therapy for such diseases. Here we summarize the characteristics of patient-specific immortalized lymphoblastoid cells, fibroblastoid cells, cytoplasmic hybrid (cybrid) cell lines, and induced pluripotent stem cells (iPSCs)-derived differentiation cells in the study of mitochondrial disorders, as well as offering discussion of roles and advances of these cell models, particularly in the screening of drugs.



Darkslateblue:Affiliate; Royal Blue:Author; Turquoise:Article


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