N-CARBAMYL-L-GLUTAMIC ACID

Absorption

30% bioavailability; Cmax, mean, 100 mg/kg dose = 2.6 μg/mL (range of 1.9 - 4.8) Carglumic acid is not subject to to intracellular degradation.

Toxicity

LD50, oral, mouse: >1000 mg/kg

The Uses of N-CARBAMYL-L-GLUTAMIC ACID

N-Carbamyl-L-glutamic Acid has been shown to have protective effects against ammonia intoxication and is used in the treatment of acute hyperammonemia.

The Uses of N-CARBAMYL-L-GLUTAMIC ACID

carbamylphosphate synthetase activator

Background

Carglumic 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. Carglumic acid was approved by the U.S. Food and Drug Administration (FDA) on 18 March 2010.

Indications

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.

What are the applications of Application

N-Carbamyl-L-glutamic Acid is a CAD complex protein activator

Definition

ChEBI: A urea that is the N-carbamoyl derivative of L-glutamic acid. An orphan drug used to treat a deficiency in the enzyme N-acetylglutamate synthase, which leads to acute hyperammonaemia.

Pharmacokinetics

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.

Metabolism

A proportion of carglumic acid may be metabolized by the intestinal bacterial flora. The likely end product of carglumic acid metabolism is carbon dioxide, eliminated through the lungs.