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[edit | edit source]
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[edit | edit source]
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[edit | edit source]
References[edit | edit source]
N-Acetylglutamate Resources | ||
---|---|---|
|
|
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
Navigation: Wellness - Encyclopedia - Health topics - Disease Index - Drugs - World Directory - Gray's Anatomy - Keto diet - Recipes
Search WikiMD
Ad.Tired of being Overweight? Try W8MD's physician weight loss program.
Semaglutide (Ozempic / Wegovy and Tirzepatide (Mounjaro / Zepbound) available.
Advertise on WikiMD
WikiMD is not a substitute for professional medical advice. See full disclaimer.
Credits:Most images are courtesy of Wikimedia commons, and templates Wikipedia, licensed under CC BY SA or similar.Contributors: Prab R. Tumpati, MD