Loss of Fas ligand-function improves survival in G93A-transgenic ALS mice Academic Article uri icon

Overview

MeSH Major

  • Amyotrophic Lateral Sclerosis
  • Fas Ligand Protein
  • Superoxide Dismutase

abstract

  • ALS is a devastating neurodegenerative disorder for which no effective treatment exists. The precise molecular mechanisms underlying the selective degeneration of motor neurons are still unknown. A motor neuron specific apoptotic pathway involving Fas and NO has been discovered. Motor neurons from ALS-mice have an increased sensitivity to Fas-induced cell death via this pathway. In this study we therefore crossed G93A-SOD1 overexpressing ALS mice with Fas ligand (FasL) mutant (gld) mice to investigate whether the reduced Fas signaling could have beneficial effects on motor neuron death. G93A-SOD1 mutant mice with a homozygous FasL mutant showed a modest but statistically significant extension of survival, and reduced loss of motor neurons. These results indicate that motor neuron apoptosis triggered by Fas is relevant in ALS pathogenesis.

publication date

  • December 21, 2006

Research

keywords

  • Academic Article

Identity

Language

  • eng

Digital Object Identifier (DOI)

  • 10.1016/j.jns.2006.08.013

PubMed ID

  • 17049562

Additional Document Info

start page

  • 44

end page

  • 9

volume

  • 251

number

  • 1-2