N-Carbamylglutamate
N-Carbamylglutamate Uses, Dosage, Side Effects, Food Interaction and all others data.
N-Carbamylglutamate 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. N-Carbamylglutamate 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 | N-Carbamylglutamate |
| 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
N-Carbamylglutamate 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.
N-Carbamylglutamate is also used to associated treatment for these conditions: Hyperammonaemia
How N-Carbamylglutamate works
N-Carbamylglutamate 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. N-Carbamylglutamate 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. N-Carbamylglutamate 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)
N-Carbamylglutamate 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
You find simplified version here N-Carbamylglutamate
