|Trade names||Carbaglu, Ucedane|
|Other names||(S)-2-ureidopentanedioic acid|
|Drug class||Carbamoyl phosphate synthetase 1 (CPS 1) activator|
|Main uses||High ammonia due to certain metabolic disorders|
|Side effects||Vomiting, abdominal pain, fever, tonsillitis, low red blood cells, diarrhea, infections, low red blood cells, low blood sugar, pancreatitis, electrolyte abnormalities, headache|
|Typical dose||10 to 250 mg/kg per day|
|US NLM||Carglumic acid|
|Elimination half-life||4.3 to 9.5 hours|
|Excretion||Fecal (60%) and kidney (9%, unchanged)|
|Chemical and physical data|
|Molar mass||190.155 g·mol−1|
|3D model (JSmol)|
|(what is this?)|
Carglumic acid, sold under the brand name Carbaglu among others, is a medication used to treat high ammonia due to N-acetylglutamate synthase (NAGS) deficiency, propionic acidemia (PA) or methylmalonic acidemia (MMA). It is taken by mouth.
Common side effects include vomiting, abdominal pain, fever, tonsillitis, low red blood cells, diarrhea, infections, low red blood cells, low blood sugar, pancreatitis, electrolyte abnormalities, and headache. Safety in pregnancy is unclear. It is a carbamoyl phosphate synthetase 1 (CPS 1) activator, which works by activating an enzyme that breaks down ammonia.
Carglumic acid was approved for medical use in Europe in 2003 and the United States in 2010. It is available as a generic medication. In the United Kingdom 60 tablets of 200 mg costs the NHS about £2,600 as of 2021. This amount in the United States costs about 13,400 USD.
- "Carbaglu- carglumic acid tablet". DailyMed. Retrieved 9 June 2021.
- "Carbaglu EPAR". European Medicines Agency (EMA). Retrieved 9 June 2021.
- "Ucedane EPAR". European Medicines Agency (EMA). Retrieved 9 June 2021.
- BNF 81: March-September 2021. BMJ Group and the Pharmaceutical Press. 2021. p. 1118. ISBN 978-0857114105.
- "Carbaglu Prices, Coupons & Patient Assistance Programs". Drugs.com. Retrieved 30 December 2021.
- "Carglumic acid Orphan Drug Designations and Approvals". U.S. Food and Drug Administration (FDA). 17 June 2014. Retrieved 9 June 2021.
- "Carglumic acid Orphan Drug Designations and Approvals". U.S. Food and Drug Administration (FDA). 20 January 1998. Retrieved 9 June 2021.
- "Carglumic acid". Drug Information Portal. U.S. National Library of Medicine.