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In addition to its role in energy production via the TCA cycle, -KG plays a central role in amino acid synthesis, redox balance, and the regulation of cell signaling

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Additionally, most patients with PNPO mutations are born prematurely and exhibit abnormal fetal movements, intrauterine distress, and amniotic fluid abnormalities in the fetus

When administered clinically, it can assist in normalizing cycles and sperm parameters