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In: Vinken PJ, Bruyn GW (eds)

For example, mutant SOD1 produces abnormal SOD molecules which are theorized to serve as the seed for large clusters of misfolded proteins that are toxic to neurons (Karch 2009

6 Extracellular acidification of gsh2 mutants is independent of l-DOPA and GSH pathway metabolites influence melanin formation

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In addition to a reduction of GPx3 levels, we also observed in our sibling cohort a large deficiency in cystine, the limiting factor to produce glutathione, which is required as a reducing agent for GPx3 and the entire glutathione peroxidase family 30,51