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

Item request has been placed! ×
Item request cannot be made. ×
loading   Processing Request
Read More Add to Saved list
  • Additional Information
    • Source:
      Publisher: Elsevier Scientific Publishers Ireland Country of Publication: Ireland NLM ID: 7600130 Publication Model: Print Cited Medium: Print ISSN: 0304-3940 (Print) Linking ISSN: 03043940 NLM ISO Abbreviation: Neurosci Lett Subsets: MEDLINE
    • Publication Information:
      Publication: Limerick : Elsevier Scientific Publishers Ireland
      Original Publication: Amsterdam, Elsevier/North-Holland.
    • Subject Terms:
      Molecular Chaperones* ; Proto-Oncogene Proteins c-bcl-2*; Carrier Proteins/*genetics ; Carrier Proteins/*metabolism ; Cell Death/*physiology ; Dystonia/*genetics ; Oxidative Stress/*physiology; Animals ; COS Cells ; Cell Survival ; Hydrogen Peroxide/toxicity ; In Situ Nick-End Labeling ; PC12 Cells ; Proto-Oncogene Proteins/metabolism ; Rats ; Reverse Transcriptase Polymerase Chain Reaction ; Transfection ; bcl-2-Associated X Protein
    • Abstract:
      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.
    • Accession Number:
      0 (Carrier Proteins)
      0 (Molecular Chaperones)
      0 (Proto-Oncogene Proteins)
      0 (Proto-Oncogene Proteins c-bcl-2)
      0 (Tor1a protein, rat)
      0 (bcl-2-Associated X Protein)
      BBX060AN9V (Hydrogen Peroxide)
    • Publication Date:
      Date Created: 20031011 Date Completed: 20031110 Latest Revision: 20191231
    • Publication Date:
      20240829
    • Accession Number:
      10.1016/s0304-3940(03)00904-2
    • Accession Number:
      14550917