期刊
BIOMOLECULES
卷 10, 期 3, 页码 -出版社
MDPI
DOI: 10.3390/biom10030450
关键词
mitochondrial disease; mitochondrial fragmentation; Drp1
资金
- MEXT/JSPS KAKENHI
- MEXT-Supported Program for the Strategic Research Foundation at Private Universities
- Uehara Memorial Foundation
- Naito Foundation
- Takeda Foundation
- Sumitomo Foundation
- Cosmetology Research Foundation
- Ono Medical Research Foundation
- Tokyo Biochemical Research Foundation
- AMED [JP17gm5010002]
- Grants-in-Aid for Scientific Research [19K16527] Funding Source: KAKEN
Mitochondria are highly dynamic organelles that constantly fuse, divide, and move, and their function is regulated and maintained by their morphologic changes. Mitochondrial disease (MD) comprises a group of disorders involving mitochondrial dysfunction. However, it is not clear whether changes in mitochondrial morphology are related to MD. In this study, we examined mitochondrial morphology in fibroblasts from patients with MD (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) and Leigh syndrome). We observed that MD fibroblasts exhibited significant mitochondrial fragmentation by upregulation of Drp1, which is responsible for mitochondrial fission. Interestingly, the inhibition of mitochondrial fragmentation by Drp1 knockdown enhanced cellular toxicity and led to cell death in MD fibroblasts. These results suggest that mitochondrial fission plays a critical role in the attenuation of mitochondrial damage in MD fibroblasts.
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