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Carnitine is naturally formed from the essential amino acids lysine and methionine and is excreted in the liver and kidneys

Whether or not (and how) glutamine-glutamate metabolism of the different isoforms dictates the fate of the metabolites further down the TCA cycle needs further investigation, as well as the potential preferential role of GAC in glutamine dependency over KGA

doi: 10.1038/nature21363 365 CahillGFJr

35 Levoketoconazole (Recorlev) is another recently approved medication for CS as of December 2021

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