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Description
Examination of electron transport chain function in muscle and fibroblast culture, histological and electron microscopy examination of muscle and other biomarkers of mitochondrial metabolism revealed a pattern consistent with the notion that PPA could be interfering with mitochondrial metabolism at the level of the tricarboxylic-acid cycle (TCAC)

This X-linked inherited disorder most commonly affects persons of African, Asian, Mediterranean, or Middle-Eastern descent

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This leads to a depletion of glutathione, a crucial molecule for protecting cells and supporting detoxification

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