Data from: Degree of glutathione deficiency and redox imbalance depend on subtype of mitochondrial disease and clinical status

Gregory M. Enns, Tereza Moore, Anthony Le, Kondala Atkuri, Monisha K. Shah, Kristina Cusmano-Ozog, Anna-Kaisa Niemi & Tina M. Cowan
Mitochondrial disorders are associated with decreased energy production and redox imbalance. Glutathione plays a central role in redox signaling and protecting cells from oxidative damage. In order to understand the consequences of mitochondrial dysfunction on in vivo redox status, and to determine how this varies by mitochondrial disease subtype and clinical severity, we used a sensitive tandem mass spectrometry assay to precisely quantify whole blood reduced (GSH) and oxidized (GSSG) glutathione levels in a large...
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