CLC number: R394.2
On-line Access: 2024-08-27
Received: 2023-10-17
Revision Accepted: 2024-05-08
Crosschecked: 2019-05-11
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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.
@article{title="Cell models and drug discovery for mitochondrial diseases",
author="Shuang-Yi Hu, Qian-Qian Zhuang, Yue Qiu, Xu-Fen Zhu, Qing-Feng Yan",
journal="Journal of Zhejiang University Science B",
volume="20",
number="5",
pages="449-456",
year="2019",
publisher="Zhejiang University Press & Springer",
doi="10.1631/jzus.B1900196"
}
%0 Journal Article
%T Cell models and drug discovery for mitochondrial diseases
%A Shuang-Yi Hu
%A Qian-Qian Zhuang
%A Yue Qiu
%A Xu-Fen Zhu
%A Qing-Feng Yan
%J Journal of Zhejiang University SCIENCE B
%V 20
%N 5
%P 449-456
%@ 1673-1581
%D 2019
%I Zhejiang University Press & Springer
%DOI 10.1631/jzus.B1900196
TY - JOUR
T1 - Cell models and drug discovery for mitochondrial diseases
A1 - Shuang-Yi Hu
A1 - Qian-Qian Zhuang
A1 - Yue Qiu
A1 - Xu-Fen Zhu
A1 - Qing-Feng Yan
J0 - Journal of Zhejiang University Science B
VL - 20
IS - 5
SP - 449
EP - 456
%@ 1673-1581
Y1 - 2019
PB - Zhejiang University Press & Springer
ER -
DOI - 10.1631/jzus.B1900196
Abstract: 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.
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