Early and Late Effects of SOD in ALS

Science  02 Jun 2006:
Vol. 312, Issue 5778, pp. 1273m
DOI: 10.1126/science.312.5778.1273m

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease primarily affecting upper and lower motor neurons and is commonly caused by toxicity resulting from dominant mutations in Cu-Zn-superoxide dismutase (SOD1). Although it appears that neuronal toxicity of SOD1 mutants is non-cell autonomous, it has yet to be established which cell types mediate damage and in which phases of the disease this takes place. Boillée et al. (p. 1389) used selective removal of a mutated SOD1 in two cell types, motor neurons or microglia-immune cells of the spinal cord, to show that the former affects timing and early disease progression, whereas the latter dictates the rate of disease progression after onset.

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