TheraRadar
Data updated: May 26, 2026

CARGLUMIC ACID

Carbamoyl Phosphate Synthetase 1 Activators
Rare Disease Approved 2021-10-13

CARGLUMIC ACID is indicated for the treatment of Hyperammonemia; N-Acetylglutamate Synthase Deficiency.

Source: FDA Label • NAVINTA LLC • Carbamoyl Phosphate Synthetase 1 Activator

How CARGLUMIC ACID Works

Carglumic acid acts as a replacement for N-acetylglutamate (NAG) by functioning as a synthetic structural analogue. NAG is the essential allosteric activator of carbamoyl phosphate synthetase 1 (CPS 1), the mitochondrial liver enzyme that catalyzes the first and rate-limiting step of the urea cycle. In patients with NAGS deficiency, the lack of endogenous NAG prevents the conversion of ammonia to urea. Carglumic acid activates CPS 1, thereby restoring the detoxification of ammonia into urea, which is then excreted in the urine.

Source: FDA Label
2
Indications
--
Phase 3 Trials
4
Years on Market

Details

Status
Prescription
First Approved
2021-10-13
Routes
ORAL
Dosage Forms
TABLET, FOR SUSPENSION

Companies

Active Ingredient: CARGLUMIC ACID

CARGLUMIC ACID Approval History

2022
2023
2024
2025
2026
Original
New Indication
New Form
Label Update
5 FDA actions from 2021 to 2024
May 2024 SUPPL
Label · Labeling
Jun 2022 ORIGINAL
Update
Oct 2021 ORIGINAL
Update

What CARGLUMIC ACID Treats

2 indications

CARGLUMIC ACID is approved for 2 conditions since its original approval in 2021. These indications span multiple therapeutic areas including oncology, immunology, and more.

  • Hyperammonemia
  • N-Acetylglutamate Synthase Deficiency
Source: FDA Label

CARGLUMIC ACID Competitive Set

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Drugs Similar to CARGLUMIC ACID

FDA-approved drugs for similar conditions. Compare mechanisms and indications to understand treatment alternatives.

CARBAGLU
CARGLUMIC ACID
2 shared
RECORDATI RARE
Shared indications:
HyperammonemiaN-Acetylglutamate Synthase Deficiency
RAVICTI
GLYCEROL PHENYLBUTYRATE
2 shared
HORIZON THERAP US
Shared indications:
HyperammonemiaN-Acetylglutamate Synthase Deficiency
SODIUM PHENYLACETATE AND SODIUM BENZOATE
SODIUM BENZOATE
1 shared
MAIA PHARMS INC
Shared indications:
Hyperammonemia
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Clinical Trial Registry

1 trials
Trial Sponsor ID Phase Status Title
NCT02426775 CAMP RC 13/116 Ph 3 completed Carglumic Acid in Methylmalonic Acidemia and Propionic Acidemia
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Active Pipeline

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Key Completed Trials

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Completed studies with published results, ranked by significance

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Trial Timeline

Full development history with FDA approval milestones

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Understanding FDA Approval Types
Count Type What it means
- ORIG Original approval - drug first enters market
- SUPPL - Efficacy New indication (new disease/condition approved)
- SUPPL - Labeling Label text changes (warnings, dosing updates)
- SUPPL - Manufacturing Production changes (new facility)
- SUPPL - Chemistry Formulation changes (new dosage strength)

Green lines in the timeline show ORIG and Efficacy approvals - the clinically meaningful milestones.

CARGLUMIC ACID FDA Label Details

Indications & Usage

CARGLUMIC ACID is indicated for the treatment of Hyperammonemia; N-Acetylglutamate Synthase Deficiency.

Looking for the branded version?

CARBAGLU

Full clinical data, patents, trials, and competitive landscape for carglumic acid.

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Data Sources

Data sourced from official FDA and NIH databases. Click links to verify on original sources.