Arginine carglumate

Amino-acidArginine derivative

What is it

Arginine carglumate is a less common pairing of the amino acid arginine with carglumic acid (N-carbamoyl-L-glutamate), a prescription drug used for rare urea cycle disorders. Genuine arginine + carglumic acid combinations should be prescribed and monitored.

Evidence for 1 use

AI-assisted evidence assessment — talk to your doctor before relying on any single supplement.

Hyperammonemia in NAGS deficiency

Good Evidence

Carglumic acid has FDA and EMA approval for treatment of hyperammonemia due to N-acetylglutamate synthase deficiency.

How it works

Carglumic acid is an analog of N-acetylglutamate, an essential cofactor for the urea cycle enzyme carbamoyl phosphate synthetase 1. It is used to treat hyperammonemia in N-acetylglutamate synthase deficiency and certain organic acidurias. Arginine is also commonly supplemented in urea cycle disorders to support nitrogen handling. The combination is a specialized medical intervention, not a general dietary supplement.

Dosage

Carglumic acid is dosed by a metabolic specialist based on weight and ammonia level (often 100 to 250 mg/kg/day in divided doses). Arginine dosing is similarly individualized.

When and how to take it

Doses are typically divided through the day per metabolic specialist instructions and taken before meals.

1 commercial form

Compare the main delivery options and what they’re best suited for.

Carglumic acid (Carbaglu)

Prescription tablet for dispersion in water, used in metabolic disorders.

Oral; dosing individualized by ammonia response.

Safety

Generally tolerated when used appropriately for urea cycle disorders. Reported effects include vomiting, abdominal pain, fever, and infections. Long-term safety is being characterized as the drug is a rare-disease medication.

Who should be cautious

Should not be used as a general supplement. Indications are limited to specific urea cycle and organic acid disorders under specialist care.

Interactions

Interacts with other ammonia-affecting medications, valproate, and high-protein supplements. Clinical management requires regular ammonia monitoring.

Frequently asked questions

What is arginine carglumate used for?⌄

The carglumic acid component is used for rare urea cycle and organic acid disorders. Arginine often accompanies it as part of medical nutrition therapy.

Can I buy it over the counter?⌄

No. Carglumic acid is a prescription medication for metabolic disorders. Self-treatment is not appropriate.

References

Arginine carglumate on NIH DSLD (US supplement label database) — NIH Dietary Supplement Label Database link

Research on Arginine carglumate (PubMed search) — PubMed link

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Evidence-based·How we grade evidence

Disclaimer: These statements have not been evaluated by the FDA. This page is educational, not a substitute for personalized medical advice. Evidence grades are AI-assisted assessments — talk to your doctor before starting any new supplement, especially if you’re pregnant, breastfeeding, on medications, or managing a chronic condition.