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The TDP-43 Q331K mouse model used in this study may be more representative of ALS patients and also shows signs of having an FTD-like phenotype with remarkable similarities to a more physiological knock-in model 25

The 1st glutathione-interaction site should be frequently (although transiently) occupied by GSH at millimolar concentrations, whereas diverse non-glutathione disulfide substrates are far less abundant in vivo and should encounter the active site less frequently