TorsinA protects against oxidative stress in COS-1 and PC12 cells

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• 作者：Kuner ; Rohini ; Teismann ; Peter ; Trutzel ; Annette ; Naim ; Jomana ; Richter ; Angelika ; Schmidt ; Nicole ; et. al.
• 关键词：Dystonia ; TorsinA ; Oxidative stress
• 刊名：Neuroscience Letters
• 出版年：2003
• 出版时间：October 30, 2003
• 年：2003
• 卷：350
• 期：3
• 页码：153-156
• 全文大小：164 K

文摘

Dystonia is a highly frequent movement disorder, the pathogenesis of which remains unclear. The cloning of TorsinA, the gene responsible for early-onset dystonia, was a major breakthrough. However, the function of this protein remains unclear. By sequence homology, TorsinA belongs to the ATPases associated with diverse cellular activities-family, many of whose members are chaperones and/or proteases. We report here that in an in vitro model for oxidative stress, H₂O₂ treatment, overexpression of TorsinA was protective against cell death. COS-1 cells overexpressing TorsinA demonstrated drastically reduced terminal deoxynucleotidyl transferase biotin-dUTP nick end labeling-staining following exposure to H₂O₂. Furthermore, transfection with TorsinA significantly increased survival of PC12 after H₂O₂ treatment. To our knowledge, this is the first demonstration that TorsinA protects against oxidative stress. We speculate that a loss of this cellular function in mutant TorsinA may be linked to the pathogenesis of early-onset dystonia.