ATC Group: A16AA Amino acids and derivatives

Ademetionine is a naturally occurring amino acid present in virtually all body tissues and fluids. Ademetionine functions primarily as a co-enzyme and donor transfer of the methyl group (transmethylation) is an essential metabolic process in humans and animals. Ademetionine is also a precursor in the formation of physiological sulfurated compounds (cysteine, taurine, glutathione, CoA, etc.) via transsulfuration. It is used for the treatment of intrahepatic cholestasis.

Betaine anhydrous was shown to lower plasma homocysteine levels in the three types of homocystinuria, i.e. CBS deficiency; MTHFR deficiency and cbl defect. The extent of this effect was dependent on the absolute degree of hyperhomocysteinemia, being higher in severe hyperhomocysteinemia. Betaine anhydrous acts as a methyl group donor in the remethylation of homocysteine to methionine in patients with homocystinuria.

Carglumic acid is a carbamoyl phosphate synthetase 1 (CPS 1) allosteric modulator. CPS1 is found in the mitochondria and is the first enzyme of the urea cycle, which converts ammonia into urea. Carglumic acid acts as a replacement for N-acetylglutamate (NAG) in N-acetylglutamate synthase (NAGS) deficiency patients by activating CPS1 but it does not help to regulate the urea cycle. Carglumic acid indicated as adjunctive therapy for the treatment of acute hyperammonemia due to the deficiency of the hepatic enzyme NAGS.

Levocarnitine as a factor is necessary in the transport of long-chain fatty acids into the mitochondria-facilitating the oxidation of fatty acids rather than their incorporation into triglycerides.

Mercaptamine reduces corneal cystine crystal accumulation acting as a cystine-depleting agent by converting cystine to cysteine and cysteine-cysteamine mixed disulfides.

Metreleptin mimics the physiological effects of leptin by binding to and activating the human leptin receptor, which belongs to the Class I cytokine family of receptors that signals through the JAK/STAT transduction pathway.