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According to this scenario, a catalytically inactive SOD1 mutant may act as a prion-like transmitter of the misfolding signal to efficiently spread the pathology, whereas the neurotoxic effect per se is caused by the ubiquitous SOD1 WT , the structural and catalytic properties of which compromised by its interaction with the misfolded SOD1 mutant

10.1038/emboj.2012.165 23 Kretz-RemyC.MehlenP.MiraultM

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The effect of microbial metabolites from colonic protein fermentation on bacteria-induced cytokine production in dendritic cells