title: Mitochondrial dysfunction in human TDP-43 transfected NSC34 cell lines and the protective effect of dimethoxy curcumin.
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.
authors.affiliation: Department of Neurology, The Second Hospital of Hebei Medical University, Hebei, China.
authors.forename: Jinsheng
authors.initials: J
authors.lastname: Lu
authors.forename: Weisong
authors.initials: W
authors.lastname: Duan
authors.forename: Yansu
authors.initials: Y
authors.lastname: Guo
authors.forename: Hong
authors.initials: H
authors.lastname: Jiang
authors.forename: Zhongyao
authors.initials: Z
authors.lastname: Li
authors.forename: Jing
authors.initials: J
authors.lastname: Huang
authors.forename: Kun
authors.initials: K
authors.lastname: Hong
authors.forename: Chunyan
authors.initials: C
authors.lastname: Li
mesh_terms: D002460:Cell Line; D003474:Curcumin / Q000031:analogs & derivatives* / Q000494:pharmacology; D019610:Cytoprotection / Q000187:drug effects* / Q000502:physiology; D004268:DNA-Binding Proteins / Q000096:biosynthesis* / Q000235:genetics; D006801:Humans; D008928:Mitochondria / Q000187:drug effects* / Q000378:metabolism* / Q000648:ultrastructure; D014162:Transfection / Q000379:methods
chemical_list: D004268:DNA-Binding Proteins; C521105:dimethoxycurcumin; D003474:Curcumin