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Yamanaka, Koji; Chun, Seung Joo; Boillee, Severine; Fujimori-Tonou, Noriko; Yamashita, Hirofumi; Gutmann, David H.; Takahashi, Ryosuke; Misawa, Hidemi; Cleveland, Don W.
Nature neuroscience, 02/2008, Letnik: 11, Številka: 3Journal Article
Dominant mutations in superoxide dismutase cause amyotrophic lateral sclerosis (ALS), an adult-onset neurodegenerative disease characterized by 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 to slow progression of non-cell-autonomous killing of motor neurons in ALS.
