Monograph
A16AA05 - Carglumic Acid |
Not porphyrinogenic |
NP |
Rationale
Non-Cyp metabolism of carbamoylated aminoacid. No conceivable physiological effects or side effects. Several indications relate to newborn or prepubertal children.
Chemical description
Carbamoyl glutamic acid. Structural analogue to N-acetyl glutamic acid, the physiological activator of hepatic carbamoylfosfate syntetase.
Therapeutic characteristics
Activator of hepatic acetylglutamate syntetase, the initial enzyme in urea biosynthesis. Under conditions of hyperammonemia the levels of ammoniac decreases and those of urea increases.
Hepatic exposure
Significant.
Metabolism and pharmacokinetics
About 30 % of the dose is absorbed giving a plasma concentration of 2-5 ug/ml. Partly metabolized to glutamic acid, and to carbon dioxide eliminated by the lungs.
Similar drugs
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