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N237S) disorder characterized by an increase in reactive oxygen species (ROS), chronic loss of axons and progressive loss of nervous system function in pediatric patients

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Eventually, GABA is removed from the system by converting it into succinate, which is then used in the Krebs cycle in the mitochondria to make ATP.[ref] The enzyme succinic semialdehyde dehydrogenase ( ALDH5A1 gene) is needed to convert GABA into succinate.[ref] Rare genetic mutations in the ALDH5A1 gene cause succinic semialdehyde dehydrogenase deficiency, which decreases the breakdown of GABA