Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

Koji Yamanaka, Seung Joo Chun, Severine Boillee, Noriko Fujimori-Tonou, Hirofumi Yamashita, David H. Gutmann, Ryosuke Takahashi, Hidemi Misawa, Don W. Cleveland

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743 Citations (Scopus)

Abstract

Dominant mutations in superoxide dismutase cause amyotrophic lateral sclerosis (ALS), an adult-onset neurodegenerative disease that is characterized by the loss of motor neurons. Using mice carrying a deletable mutant gene, diminished mutant expression in astrocytes did not affect onset, but delayed microglial activation and sharply slowed later disease progression. These findings demonstrate that mutant astrocytes are viable targets for therapies for slowing the progression of non-cell autonomous killing of motor neurons in ALS.

Original languageEnglish
Pages (from-to)251-253
Number of pages3
JournalNature Neuroscience
Volume11
Issue number3
DOIs
Publication statusPublished - 2008 Mar 1

ASJC Scopus subject areas

  • Neuroscience(all)

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  • Cite this

    Yamanaka, K., Chun, S. J., Boillee, S., Fujimori-Tonou, N., Yamashita, H., Gutmann, D. H., Takahashi, R., Misawa, H., & Cleveland, D. W. (2008). Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis. Nature Neuroscience, 11(3), 251-253. https://doi.org/10.1038/nn2047