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Abstract Title:

Mitochondrial dysfunction in human TDP-43 transfected NSC34 cell lines and the protective effect of dimethoxy curcumin.

Abstract Source:

Brain Res Bull. 2012 Dec 1 ;89(5-6):185-90. Epub 2012 Sep 15. PMID: 22986236

Abstract Author(s):

Jinsheng Lu, Weisong Duan, Yansu Guo, Hong Jiang, Zhongyao Li, Jing Huang, Kun Hong, Chunyan Li

Article Affiliation:

Jinsheng Lu

Abstract:

TAR-DNA-binding protein of 43kDa (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.

Study Type : In Vitro Study

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Sayer Ji
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