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The mutation in ALDH4A1 leads to the accumulation of pyrroline-5-carboxylate (P5C) in the mitochondria, which can react with PLP to cause PLP deficiency in the brain and can also be converted to proline, resulting in elevated plasma proline levels (Fig

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According to the NIH Office of Dietary Supplements, vitamin B12 is available as cyanocobalamin and hydroxocobalamin, which are typically administered parenterally by intramuscular injection to bypass gastrointestinal absorption barriers