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Deletion of Mutant Astrocyte Gene Delays ALS Progression

Also delays microglial activation in mouse study of amyotrophic lateral sclerosis

THURSDAY, Feb. 7 (HealthDay News) -- Reducing the expression of a mutant gene that causes amyotrophic lateral sclerosis (ALS) specifically in astrocytes in mice slows later disease progression and delays microglial activation, according to a report published online Feb. 3 in Nature Neuroscience.

Koji Yamanaka, M.D., Ph.D., from the RIKEN Brain Science Institute in Saitama, Japan, and colleagues generated mice with a mutant superoxide dismutase gene that could be selectively deleted in the spinal cord and in astrocytes.

The researchers found that reduced expression of the mutant gene in astrocytes did not slow disease onset, but sharply delayed late disease progression from early disease to end stage. Overall survival was extended by 60 days. Microglial activation was also substantially delayed from onset through early disease.

"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," Yamanaka and colleagues conclude.

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