. Loss of ALS2 function is insufficient to trigger motor neuron degeneration in knock-out mice but predisposes neurons to oxidative stress. J Neurosci. 2005 Aug 17;25(33):7567-74. PubMed.


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  1. It is now well-appreciated that so many of the models in mice that recapitulate the mutations that cause dominant, fully penetrant neurodegeneration in humans have such mild phenotypes in mice. Here, the authors find another such mild phenotype—susceptibility to oxidative stress—which provides a potential pathophysiological link to other forms of ALS. This is intriguing in that work in human and nonhuman primates now suggests that gene expression in general, and specifically oxidative metabolism genes, are increased in humans. Increased susceptibility to neurodegeneration may be the price that one pays for having a metabolically more active brain—a factor that needs to be clearly considered in animal models of any type. Further exploration of this and other models will help to clarify the likely central role of stress.

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