Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis.
Journal:
Nat. Neurosci. 2008 MarAuthors:
Yamanaka K, Chun SJ, Boillee S, Fujimori-Tonou N, Yamashita H, Gutmann DH, Takahashi R, Misawa H, Cleveland DW
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 autonomo
...[more]us killing of motor neurons in ALS.[less]
Mesh Headings:
Amyotrophic Lateral Sclerosis, Animals, Astrocytes, Cell Death, Disease Models, Animal, Disease Progression, Genes, Dominant, Genetic Therapy, Glial Fibrillary Acidic Protein, Gliosis, Humans, Integrases, Mice, Mice, Transgenic, Microglia, Mutation, Nerve Degeneration, Nitric Oxide, Nitric Oxide Synthase Type II, Oxidative Stress, Spinal Cord, Superoxide Dismutase, Survival Rate