Journal of Molecular Neuroscience

, Volume 38, Issue 2, pp 94–102 | Cite as

DJ-1 Changes in G93A-SOD1 Transgenic Mice: Implications for Oxidative Stress in ALS

  • Nirit Lev
  • Debby Ickowicz
  • Yael Barhum
  • Eldad Melamed
  • Daniel Offen
Article

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive, lethal, neurodegenerative disorder. The causes of ALS are still obscure. Accumulating evidence supports the hypothesis that oxidative stress and mitochondrial dysfunction can be implicated in ALS pathogenesis. DJ-1 plays an important role in the oxidative stress response. The aim of this study was to discover whether there are changes in DJ-1 expression or in DJ-1-oxidized isoforms in an animal model of ALS. We used mutant SOD1G93A transgenic mice, a commonly used animal model for ALS. Upregulation of DJ-1 mRNA and protein levels were identified in the brains and spinal cords of SOD1G93A transgenic mice as compared to wild-type controls, evident from an early disease stage. Furthermore, an increase in DJ-1 acidic isoforms was detected, implying that there are more oxidized forms of DJ-1 in the CNS of SOD1G93A mice. This is the first report of possible involvement of DJ-1 in ALS. Since DJ-1 has a protective role against oxidative stress, it may suggest a possible therapeutic target in ALS.

Keywords

Amyotrophic lateral sclerosis (ALS) DJ-1 Cu/Zn superoxide dismutase (SOD1) Oxidative stress 

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Copyright information

© Humana Press 2008

Authors and Affiliations

  • Nirit Lev
    • 1
  • Debby Ickowicz
    • 1
  • Yael Barhum
    • 1
  • Eldad Melamed
    • 1
  • Daniel Offen
    • 1
  1. 1.Laboratory of Neurosciences, Felsenstein Medical Research Center and Department of Neurology, Rabin Medical Center-Beilinson Campus, The Sackler School of MedicineTel Aviv UniversityPetah TikvaIsrael

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