N-Acetylglutamate

N-Acetylglutamate (NAG) is a molecule that plays a crucial role in the urea cycle, a process that helps the body eliminate excess nitrogen. NAG is produced in the mitochondria of liver cells and is the first compound in the urea cycle. It acts as a necessary enzyme activator for the process to proceed.

Structure and Function

NAG is a derivative of glutamate, an important amino acid in the body. It is formed when an acetyl group is added to glutamate in a reaction catalyzed by the enzyme N-acetylglutamate synthase (NAGS).

The primary function of NAG is to activate the enzyme carbamoyl phosphate synthetase I (CPS1), which is the first enzyme in the urea cycle. Without NAG, CPS1 cannot function, and the urea cycle cannot proceed. This can lead to a buildup of nitrogen in the body, which can be harmful.

Clinical Significance

Deficiencies in NAG can lead to a variety of health problems. The most severe is N-acetylglutamate synthase deficiency, a rare genetic disorder that prevents the body from producing enough NAG. This can lead to a buildup of ammonia in the blood, a condition known as hyperammonemia. Symptoms can include poor growth, intellectual disability, and life-threatening health problems.

NAG is also used in the treatment of some genetic disorders of the urea cycle. For example, it can be used to treat carbamoyl phosphate synthetase I deficiency, a condition that prevents the body from removing nitrogen from the blood.

See Also

• Urea cycle
• Glutamate
• Hyperammonemia
• Carbamoyl phosphate synthetase I deficiency

References

N-Acetylglutamate Resources
Need Help Finding a Doctor? Need help finding a doctor or specialist anywhere in the world? Explore our world directory. WikiMD's DocFinder can assist you in locating millions of physicians.	Medical Knowledge Access the latest research - Most recent articles Ongoing trials.

Translate to: East Asian 中文, 日本, 한국어, South Asian हिन्दी, Urdu, বাংলা, తెలుగు, தமிழ், ಕನ್ನಡ,
Southeast Asian Indonesian, Vietnamese, Thai, မြန်မာဘာသာ, European español, Deutsch, français, русский, português do Brasil, Italian, polski