期刊
BRAIN RESEARCH BULLETIN
卷 89, 期 5-6, 页码 185-190出版社
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.brainresbull.2012.09.005
关键词
ALS; Dimethoxy curcumin; Electron transport chain; Mitochondrial transmembrane potential; TDP-43; UCP2
资金
- National Natural Science Foundation of China
- Hebei Science and Technology Department [30870882, 30900460, 11966122D]
TAR-DNA-binding protein of 43 kDa (TDP-43) was recently found to be one of the major disease proteins in the pathological inclusions of amyotrophic lateral sclerosis (ALS). The effect of TDP-43 on mitochondrial function remains poorly understood. Here, we show that human TDP-43 caused mitochondrial morphologic abnormality, decrease of mitochondrial complex I activity and mitochondrial transmembrane potential, and increased expression of mitochondrial uncoupling protein 2 (UCP2) in human TDP-43 stably transfected NSC-34 cells by using flow cytometric analysis, spectrophotometric assays, electron microscopy and Western blotting. We also show that dimethoxy curcumin (DMC) could ameliorate mitochondrial dysfunction in mutated TDP-43 stably transfected cell lines. DMC could be potentially useful for neurodegenerative diseases linked with mutated TDP-43. (C) 2012 Elsevier Inc. All rights reserved.
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