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Decreased glutathione accelerates neurological deficit and mitochondrial pathology in familial ALS-linked hSOD1(G93A) mice model

These treatment conditions were chosen to mimic excess heme from chronic hemolysis and systemic hypoxic conditions that occur in SCD patients 1,2,11

Since D2HG and L2HG are indistinguishable in standard LC-MS, to determine which 2HG enantiomer(s) is affected, we adapted a chiral N-(p-toluenesulfonyl)-L-phenylalanyl chloride (TSPC) derivation followed by liquid chromatography with tandem mass spectrometry (LC-MS/MS) analysis 24,40

71st Annual Meeting of the Canadian Society of Otolaryngology Head and Neck Surgery, US

All treatments were well tolerated (Extended Data Fig