Underediting of GluR2 mRNA, a neuronal death inducing molecular change in sporadic ALS, does not occur in motor neurons in ALS1 or SBMA

Yukio Kawahara, Hui Sun, Kyoko Ito, Takuto Hideyama, Masashi Aoki, Gen Sobue, Shoji Tsuji, Shin Kwak

Research output: Contribution to journalArticlepeer-review

59 Citations (Scopus)

Abstract

Deficient RNA editing of the AMPA receptor subunit GluR2 at the Q/R site is a primary cause of neuronal death and recently has been reported to be a tightly linked etiological cause of motor neuron death in sporadic amyotrophic lateral sclerosis (ALS). We quantified the RNA editing efficiency of the GluR2 Q/R site in single motor neurons of rats transgenic for mutant human Cu/Zn-superoxide dismutase (SOD1) as well as patients with spinal and bulbar muscular atrophy (SBMA), and found that GluR2 mRNA was completely edited in all the motor neurons examined. It seems likely that the death cascade is different among the dying motor neurons in sporadic ALS, familial ALS with mutant SOD1 and SBMA.

Original languageEnglish
Pages (from-to)11-14
Number of pages4
JournalNeuroscience Research
Volume54
Issue number1
DOIs
Publication statusPublished - 2006 Jan

Keywords

  • ALS
  • AMPA receptor
  • GluR2
  • Motor neuron
  • Neuronal death
  • RNA editing
  • SOD1
  • Spinal and bulbar muscular atrophy

ASJC Scopus subject areas

  • Neuroscience(all)

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