Carglumic acid

Structural formula
General
Surname	Carglumic acid
other names	N -carbamyl-L-glutamic acid
Molecular formula	C 6 H 10 N 2 O 5
External identifiers / databases
CAS number 1188-38-1 EC number 601-569-3 ECHA InfoCard 100.116.323 PubChem 121396 ChemSpider 108351 DrugBank DB06775 Wikidata Q822884
Drug information
ATC code	A16 AA05
properties
Molar mass	190.15 g · mol -1
safety instructions
Please note the exemption from the labeling requirement for drugs, medical devices, cosmetics, food and animal feed GHS labeling of hazardous substances Caution H and P phrases H: 302-315-319-335 P: 261-305 + 351 + 338
Toxicological data	1000 mg kg −1 ( LD 50 ,  mouse ,  oral )
As far as possible and customary, SI units are used. Unless otherwise noted, the data given apply to standard conditions .

Carglumic acid is an orphan drug used to treat hyperammonaemia caused by deficiency in N- acetylglutamate synthetase . The chemical compound is derived from carbamic acid and glutamic acid and, like the latter, is chiral .

pharmacology

Potentially brain-damaging hyperammonaemia occurs when the urea cycle in the body no longer works, i.e. the body cannot detoxify ammonia by incorporating it into urea . This is the case with a deficiency of N- acetylglutamate synthetase. The cause is mutations in the NAGS gene ( chromosome region 17q21.31 ). The product of N -acetylglutamate synthetase ( N -acetylglutamate ) serves as an allosteric activator for carbamoyl phosphate synthetase I in the body .

Carglumic acid is a structural analogue of the missing N -acetylglutamate and can also serve as an activator. It works even better therapeutically.

Individual evidence

1. ↑ ^{a b c} Data sheet N-Carbamyl-L-glutamic acid from Sigma-Aldrich , accessed on June 23, 2014 ( PDF ).
2. ↑ ^{a b} H. Lüllmann, K. Mohr, L. Hein: Pharmakologie und Toxikologie , 17th edition, Georg Thieme Verlag, Stuttgart 2010, ISBN 978-3-13-151647-3 , p. 267.