Early decrease of mitochondrial DNA repair enzymes in spinal motor neurons of presymptomatic transgenic mice carrying a mutant SOD1 gene

Tetsuro Murakami, Makiko Nagai, Kazunori Miyazaki, Nobutoshi Morimoto, Yasuyuki Ohta, Tomoko Kurata, Yasushi Takehisa, Tatsushi Kamiya, Koji Abe

Research output: Contribution to journalArticlepeer-review

30 Citations (Scopus)

Abstract

Growing evidence has recently shown that mutant SOD1 accumulate in the mitochondria and cause vacuolation in transgenic mice carrying mutant SOD1, an animal model of amyotrophic lateral sclerosis (ALS). In this study, the expressions of DNA repair enzymes, oxoguanine glycosylase 1 (ogg1), DNA polymerase β (polβ), and DNA polymerase γ (polγ) were examined in transgenic mice with an ALS-linked mutant SOD1 gene, a valuable model for human ALS. In presymptomatic Tg mice, the nuclear form of ogg1 was upregulated, whereas mitochondrial ogg1 remained at the same level. DNA polymerase was selectively downregulated in the mitochondria. This study suggests an impaired protective mechanism against oxidative stress in mitochondria. The expressions of these enzymes are predominant in spinal motor neurons, suggesting a mechanism of selective motor neuron death in this animal model of ALS.

Original languageEnglish
Pages (from-to)182-189
Number of pages8
JournalBrain Research
Volume1150
Issue number1
DOIs
Publication statusPublished - Aug 31 2007

ASJC Scopus subject areas

  • Neuroscience(all)
  • Molecular Biology
  • Clinical Neurology
  • Developmental Biology

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