. Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset. PLoS One. 2011;6(7):e22031. PubMed.


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  1. My quick impression is that this work by Vande Velde et al. confirms many earlier studies suggesting that mitochondrial pathobiology may have an important role in the pathogenesis of ALS in mouse models. It is exciting and encouraging that many of the images published in this paper are very similar to previously published work originating for the Xu, Bendotti, and our own lab. Important unanswered questions are, How generalizable is the mitochondrial pathobiology mechanism in other mouse models of ALS, and are similar events occurring in human ALS? We can only hope that mitochondria do have an important role in human ALS, and that mitochondrial-targeted drugs, such as olesoxime, which is in clinical trials in the EU, have a significant beneficial effect.

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