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One is direct action in the kidney by stimulation of renal glutaminase, and indirect action in the liver by inhibition of NAGS, which reduces the availability of NAG, a cofactor required for the activity of carbamoyl phosphate synthetase (CPS I), the first enzyme in the urea cycle.5 They also cause carnitine depletion, affecting the beta-oxidation of fatty acids and resulting in decreased levels of acetyl-CoA, which is necessary for NAG synthesis11 (Fig

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