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This metabolic transition can manifest as fatigue, especially in the first few weeks of treatment
In addition, GAMT deficiency requires arginine restriction through a low-protein diet, and ornithine supplementation and benzoate (reduces glycine levels via the formation and excretion of hippuric acid) to reduce guanidinoacetate synthesis and its associated toxicity (creatine supplementation also partially inhibits guanidinoacetate formation 4,5 )
Wolfson, R.L., et al.,Sestrin2 is a leucine sensor for the mTORC1 pathway
These medications work best when a clinician understands the full picture, not just a number on a form