Carglumic Acid
Amino Acids and Derivatives
Sold as Ucedane · Carbaglu
- Drug class
- Amino Acids and Derivatives
- Availability
- Prescription only
- Routes
- Oral
- Markets covered
- 4
- Products on record
- 7
- FDA reports (12 mo)
- 38
Overview
Carglumic Acid is an active pharmaceutical ingredient in the Amino Acids and Derivatives group (A16AA). The information below is compiled per regulator from the product labels on record, with direct links to the original documents.
Regulatory status by market
| Market | Regulator | Products | Last revision |
|---|---|---|---|
| GB United Kingdom | MHRA | 3 | May 29, 2026 |
| EU European Union | EMA | 2 | May 26, 2026 |
| US United States | FDA | 1 | January 25, 2024 |
| CA Canada | Health Canada | 1 | March 22, 2025 |
GBUnited Kingdom· MHRA
3 products
Uses
Carbaglu is indicated in treatment of • hyperammonaemia due to N-acetylglutamate synthase primary deficiency. • hyperammonaemia due to isovaleric acidaemia. • hyperammonaemia due to methymalonic acidaemia. • hyperammonaemia due to propionic acidaemia.
How to take
EUEuropean Union· EMA
2 products
Uses
Ucedane is indicated in treatment of • hyperammonaemia due to N-acetylglutamate synthase primary deficiency. • hyperammonaemia due to isovaleric acidaemia. • hyperammonaemia due to methymalonic acidaemia. • hyperammonaemia due to propionic acidaemia.
How to take
USUnited States· FDA
1 product
Uses
1. INDICATIONS AND USAGE CARBAGLU is a carbamoyl phosphate synthetase 1 (CPS 1) activator indicated in pediatric and adult patients as: Adjunctive therapy to standard of care for the treatment of acute hyperammonemia due to N-acetylglutamate synthase (NAGS) deficiency.
1 ) Maintenance therapy for the treatment of chronic hyperammonemia due to NAGS deficiency. 1 ) Adjunctive therapy to standard of care for the treatment of acute hyperammonemia due to propionic acidemia (PA) or methylmalonic acidemia (MMA).
1 Acute and Chronic Hyperammonemia due to N‑acetylglutamate Synthase (NAGS) Deficiency CARBAGLU is indicated in adult and pediatric patients as: Adjunctive therapy to standard of care for the treatment of acute hyperammonemia due to NAGS deficiency.
Maintenance therapy for the treatment of chronic hyperammonemia due to NAGS deficiency. 2 Acute Hyperammonemia due to Propionic Acidemia (PA) or Methylmalonic Acidemia (MMA) CARBAGLU is indicated in adult and pediatric patients as adjunctive therapy to standard of care for the treatment of acute hyperammonemia due to PA or MMA.
CACanada· Health Canada
1 product
Uses
CARBAGLU (Carglumic acid dispersible tablets) is indicated for: • Acute hyperammonemia in patients with NAGS deficiency: CARBAGLU is indicated as an adjunctive therapy in pediatric and adult patients for the treatment of acute hyperammonemia due to the deficiency of the hepatic enzyme N-acetylglutamate synthase (NAGS).
During acute hyperammonemic episodes, concomitant administration of CARBAGLU with other ammonia lowering therapies such as alternate pathway medications, hemodialysis, and dietary protein restriction are recommended. • Maintenance therapy for chronic hyperammonemia in patients with NAGS deficiency: CARBAGLU is indicated for maintenance therapy in pediatric and adult patients for chronic hyperammonemia due to the deficiency of the hepatic enzyme N- acetylglutamate synthase (NAGS).
During maintenance therapy, the concomitant use of other ammonia lowering therapies and protein restriction may be reduced or discontinued based on plasma ammonia levels. • Acute hyperammonemia due to Propionic Acidemia (PA): CARBAGLU is indicated, in pediatric and adult patients, for the treatment of acute hyperammonemic episodes due to propionic acidemia (PA), as an adjunctive treatment to other ammonia lowering therapies.
Sources & citations
- [1]MHRA (UK) · PLGB152660019 · revised April 10, 2026
- [2]European Medicines Agency · EMEA/H/C/004019 · revised May 26, 2026
- [3]FDA DailyMed · 10175e73-5172-4d… · revised January 25, 2024 [PDF]
- [4]Health Canada (DPD) · 02439360 · revised March 22, 2025
- [5]OpenFDA adverse-event reports (US), 12 months ending June 4, 2026.
Information on this page is compiled from public regulatory records. Drugvu is not affiliated with any regulator or pharmaceutical manufacturer. This is not medical advice. Always consult a qualified healthcare professional.