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Nat Neurosci. 2008 Mar;11(3):251-3. doi: 10.1038/nn2047. Epub 2008 Feb 3.

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis.

Author information

1
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.

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.

PMID:
18246065
PMCID:
PMC3137510
DOI:
10.1038/nn2047
[Indexed for MEDLINE]
Free PMC Article

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