Carbamino-L-glutamic Acid
Carbamino-L-glutamic Acid Uses, Dosage, Side Effects, Food Interaction and all others data.
Carbamino-L-glutamic Acid is a drug used for the treatment of hyperammonemia in patients with a deficiency in N-acetyl glutamate synthase. This rare genetic disorder results in elevated blood levels of ammonia, which can eventually cross the blood–brain barrier and cause neurologic problems, cerebral edema, coma, and death. Carbamino-L-glutamic Acid was approved by the U.S. Food and Drug Administration (FDA) on 18 March 2010.
The median Tmax of Carbaglu was 3 hours (range: 2-4). The daily dose of carglumic acid ranges from 100 to 250 mg/kg and this does are normally adjusted to maintain normal plasma levels of ammonia.
| Trade Name | Carbamino-L-glutamic Acid |
| Generic | Carglumic acid |
| Carglumic acid Other Names | Acide carglumique, ácido carglúmico, Acidum carglumicum, Carbamino-L-glutamic acid, Carbamylglutamic acid, Carglumic acid, N-Carbamylglutamate, Ureidoglutaric acid |
| Type | |
| Formula | C6H10N2O5 |
| Weight | Average: 190.154 Monoisotopic: 190.05897144 |
| Groups | Approved |
| Therapeutic Class | |
| Manufacturer | |
| Available Country | |
| Last Updated: | September 19, 2023 at 7:00 am |
Uses
Carbamino-L-glutamic Acid is an analog of N-acetylglutamate (NAG) used for the treatment of acute and chronic hyperammonemia in patients with N-acetylglutamate synthase (NAGS) deficiency.
For the treatment of acute and chronic hyperammonaemia in patients with N-acetylglutamate synthase (NAGS) deficiency. This enzyme is an important component of the urea cycle to prevent build up of neurotoxic ammonium in the blood.
Carbamino-L-glutamic Acid is also used to associated treatment for these conditions: Hyperammonaemia
How Carbamino-L-glutamic Acid works
Carbamino-L-glutamic Acid is a synthetic structural analogue of N-acetylglutamate (NAG), which is an essential allosteric activator of the liver enzyme carbamoyl phosphate synthetase 1 (CPS1). CPS1 is found in the mitochondria and is the first enzyme of the urea cycle, which converts ammonia into urea. Carbamino-L-glutamic Acid acts as a replacement for NAG in NAGS deficiency patients by activating CPS1 but it does not help to regulate the urea cycle.
Toxicity
LD50, oral, mouse: >1000 mg/kg
Food Interaction
- Take before a meal. Take carglumic acid right before eating.
- Take with plain water. Carbamino-L-glutamic Acid tablets are dispersed in water to produce an oral suspension and should not be swallowed whole.
Volume of Distribution
The apparent volume of distribution was 2657 L (range: 1616-5797).
Elimination Route
30% bioavailability;
Cmax, mean, 100 mg/kg dose = 2.6 μg/mL (range of 1.9 - 4.8)
Carbamino-L-glutamic Acid is not subject to to intracellular degradation.
Half Life
Median values for the terminal half-life was 5.6 hours (range 4.3-9.5).
Clearance
The apparent total clearance was 5.7 L/min (range 3.0-9.7), the renal clearance was 290 mL/min (range 204-445), and the 24-hour urinary excretion was 4.5 % of the dose (range 3.5-7.5).
Elimination Route
Following administration of a single radiolabeled oral dose of 100 mg/kg of body weight, 9% of the dose was excreted unchanged in the urine and up to 60% of the dose was excreted unchanged in the feces.
Innovators Monograph
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