glutathione peroxidase deficiency treatment 4: a new player in neurodegeneration? Molecular Medicine Reports

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Ricci G, De Maria F, Antonini G, Turella P, Bullo A, Stella L et al

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In a model of amyotrophic lateral sclerosis (ALS) caused by C9orf72 gene mutations, the pathogenic poly-dipeptide GA50 directly binds to and inhibits SQOR, leading to excessive NLRP3 inflammasome activation in microglia and consequent mitochondrial dysfunction ( A growing body of preclinical work supports this strategy: boosting SQOR activity or reducing H 2 S can protect the nervous system