Isovaleryl-CoA
Isovaleryl-CoA is an intermediate in the metabolism of leucine, an essential amino acid in humans. It is a substrate for the enzyme isovaleryl-CoA dehydrogenase, which catalyzes the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA.
Metabolism[edit | edit source]
Leucine is an essential amino acid that is metabolized in the body to produce energy. The first step in the metabolism of leucine is the transamination of leucine to α-ketoisocaproate by the enzyme branched-chain amino acid transaminase. α-Ketoisocaproate is then decarboxylated to isovaleryl-CoA by the enzyme branched-chain alpha-keto acid dehydrogenase complex.
Isovaleryl-CoA is then converted to 3-methylcrotonyl-CoA by the enzyme isovaleryl-CoA dehydrogenase. This reaction is a part of the leucine degradation pathway, which ultimately leads to the production of acetyl-CoA, a key molecule in energy production.
Clinical significance[edit | edit source]
Mutations in the gene encoding isovaleryl-CoA dehydrogenase can lead to isovaleric acidemia, a rare metabolic disorder characterized by a deficiency of isovaleryl-CoA dehydrogenase. This can result in an accumulation of isovaleryl-CoA and its metabolites in the body, leading to various symptoms such as poor feeding, vomiting, lethargy, and in severe cases, coma or death.
See also[edit | edit source]
- Leucine
- Isovaleric acidemia
- Branched-chain amino acid transaminase
- Branched-chain alpha-keto acid dehydrogenase complex
- Isovaleryl-CoA dehydrogenase
Isovaleryl-CoA 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) 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