@article{19bc1b38baee4ee0adb2b56638e63486,
title = "Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis",
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.",
author = "Koji Yamanaka and Chun, {Seung Joo} and Severine Boillee and Noriko Fujimori-Tonou and Hirofumi Yamashita and Gutmann, {David H.} and Ryosuke Takahashi and Hidemi Misawa and Cleveland, {Don W.}",
note = "Funding Information: This work was supported by a US National Institutes of Health grant (NS 27036) and a grant from the Packard ALS Center at Johns Hopkins (D.W.C.), as well as a Muscular Dystrophy Association developmental grant, the Uehara Memorial Foundation, the Nakabayashi Trust for ALS Research and a grant-in-aid for Scientific Research (19591021) and on Priority Area (19044048) from the Ministry of Education, Culture, Sports, Science and Technology of Japan (K.Y.). Salary support for D.W.C is provided by the Ludwig Institute for Cancer Research. S.B. is a recipient of a Fondation pour la Recherche Medical fellowship, an Institut National de la sant{\'e} et de la Recherch{\'e} Medicale fellowship and a Muscular Dystrophy Association developmental grant.",
year = "2008",
month = mar,
doi = "10.1038/nn2047",
language = "English",
volume = "11",
pages = "251--253",
journal = "Nature Neuroscience",
issn = "1097-6256",
publisher = "Nature Publishing Group",
number = "3",
}