Journal
EXPERIMENTAL NEUROLOGY
Volume 207, Issue 1, Pages 107-117Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.expneurol.2007.05.026
Keywords
nFE2-related factor 2 (Nrf2); antioxidant response element (ARE); oxidative stress; amyotrophic lateral sclerosis (ALS); superoxide dismutase (SOD); motor neuron disease; muscle; neurodegeneration
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Funding
- NIEHS NIH HHS [ES10042, ES08089, R01 ES008089-10, R01 ES008089-10S1, R01 ES010042, R29 ES008089, R01 ES008089-09, R01 ES008089] Funding Source: Medline
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Oxidative stress plays a key role in the neuronal loss exhibited in amyotrophic lateral sclerosis (ALS), an event precipitating irreversible muscle atrophy. By crossing ALS mouse models (SOD (G93A) and SOD (H46RH48Q)) with an antioxidant response element (ARE) reporter mouse, we identified activation characteristics of the ARE system throughout the timecourse of motor neuron disease. Surprisingly, the earliest and most significant activation of this genetic sensor of oxidative stress occurred in the distal muscles of mutant SOD mice. The resultant data supports existing hypotheses that the muscle is somehow implicated during the initial pathology of these mice. Subsequently, Nrf2-ARE activation appears to progress in a retrograde fashion along the motor pathway. These data provide timely information concerning the contributions of the Nrf2-ARE pathway in ALS disease progression. (c) 2007 Elsevier Inc. All rights reserved.
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