Brief Communication abstract
Nature Neuroscience 11, 251 - 253 (2008)
Published online: 3 February 2008 | doi:10.1038/nn2047
Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis
Koji Yamanaka1,2, Seung Joo Chun1, Severine Boillee1, Noriko Fujimori-Tonou2, Hirofumi Yamashita2, David H Gutmann3, Ryosuke Takahashi4, Hidemi Misawa5 & Don W Cleveland1
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.
- Ludwig Institute for Cancer Research and Department of Medicine and Neuroscience, University of California at San Diego, 9500 Gilman Drive, La Jolla, California 92093-0670, USA.
- Yamanaka Research Unit, RIKEN Brain Science Institute, 2-1 Hirosawa, Wako, Saitama 351-0198, Japan.
- Department of Neurology, Washington University School of Medicine, 660 South Euclid Avenue, St. Louis, Missouri 63110, USA.
- Department of Neurology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan.
- Department of Pharmacology, Kyoritsu University of Pharmacy, 1-5-30 Shibakoen, Minato-ku, Tokyo 105-8512, Japan.
Correspondence to: Don W Cleveland1 e-mail: dcleveland@ucsd.edu
Correspondence to: Koji Yamanaka1,2 e-mail: kyamanaka@brain.riken.jp
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