期刊
JOURNAL OF ZHEJIANG UNIVERSITY-SCIENCE B
卷 20, 期 5, 页码 449-456出版社
ZHEJIANG UNIV
DOI: 10.1631/jzus.B1900196
关键词
Mitochondrial diseases; Mitochondrial DNA; Cell model; Drug discovery
类别
资金
- National Basic Research Program of China [2014CB943001]
- National Natural Science Foundation of China [31771398, 31571299]
- Fundamental Research Funds for the Central Universities [2019QNA6001]
- Zhejiang Provincial Natural Science Foundation of China [LZ19C060001, LY14C060004]
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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