Decreased glutathione accelerates neurological deficit and mitochondrial pathology in familial ALS-linked hSOD1G93A mice model

► Decreased glutathione content accelerated the disease in ALS-linked hSOD1G93A mice. ► Decreased glutathione aggravated mitochondrial pathology in hSOD1G93A mice. ► Decreased glutathione increased the association of hSOD1G93A with the mitochondria. ► Decreased glutathione content did not accelerate the disease in hSOD1H46R/H48Q mice.