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Onset and progression in inherited ALS determined by motor neurons and microglia.

Dominant mutations in superoxide dismutase cause amyotrophic lateral sclerosis (ALS), a progressive paralytic disease characterized by loss of motor neurons. With the use of mice carrying a deletable mutant gene, expression within motor neurons was shown to be a primary determinant of disease onset and of an early phase of disease progression. Diminishing the mutant levels in microglia had little effect on the early disease phase but sharply slowed later disease progression. Onset and progression thus represent distinct disease phases defined by mutant action within different cell types to generate non-cell-autonomous killing of motor neurons; these findings validate therapies, including cell replacement, targeted to the non-neuronal cells.

Pubmed ID: 16741123


  • BoillĂ©e S
  • Yamanaka K
  • Lobsiger CS
  • Copeland NG
  • Jenkins NA
  • Kassiotis G
  • Kollias G
  • Cleveland DW


Science (New York, N.Y.)

Publication Data

June 2, 2006

Associated Grants

  • Agency: Medical Research Council, Id: MC_U117581330
  • Agency: NINDS NIH HHS, Id: NS 27036
  • Agency: NINDS NIH HHS, Id: R37 NS027036

Mesh Terms

  • Amyotrophic Lateral Sclerosis
  • Animals
  • Antigens, CD11b
  • Disease Progression
  • Female
  • Humans
  • Integrases
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Microglia
  • Motor Neurons
  • Mutation
  • Superoxide Dismutase