N-acetylglutamate synthetase deficiency (NAGS)
is a rare genetic disorder caused by a deficiency in the N-acetylglutamate Synthetase enzyme.To minimize the build up of ammonia in the blood, people with NAGS are prescribed a low protein diet.
This App makes daily protein counting and meal planning easier for people following low protein diets.
What is NAGS?
N-acetylglutamate synthetase deficiency (NAGS) is a genetic disorder caused by a deficiency in the N-acetylglutamate Synthetase enzyme. Read more on NAGS via this link.
N-acetyl glutamate synthetase (NAGS) is an enzyme that helps form N-acetylglutamate (NAG) from the amino acid glutamine (Glu) and enzyme acetylcoenzyme A (CoA).
NAGS increases in activity when exposed to the amino acid arginine (Arg).
NAG is responsible for activating the first, rate-determining enzyme of the urea cycle, carbamylphosphate I (CPS I).
Due to the impact NAGS has on the urea cycle, it is part of a group of disorders known as Urea Cycle Disorders.
Arg and Glu are important amino acids that makes up protein in foods. Protein is important for growth in children and health maintenance in adults.
High levels ammonia may cause intellectual disorders.
Symptoms of NAGS may appear as early as the first few days of life. Some common NAGS symptoms are: loss of appetite, seizures, poorly controlled breathing and body temperature, as well as vomiting.
Why a diet?
To minimize the build up of ammonia in the blood, people with NAGS are prescribed a low protein diet.
People living with NAGS are prescribed their daily protein restriction by their doctor or dietitian. These daily goals often need to be changed based on each person’s nutritional needs and age requirements.
Calculating and tracking protein intake can be a very hard and troublesome task for those with protein-restricted diets.
This App makes daily protein counting and meal planning easier for people following low protein diets.
For whom?
This App is designed to support families and children with NAGS, treated with a protein-restricted diet.
This App is not a substitute for the health care provided by your medical team!