Methylmalonyl-CoA
Methylmalonyl-CoA is a molecule that plays a crucial role in human metabolism. It is involved in the breakdown and use of proteins and fats in the body. Methylmalonyl-CoA is converted into succinyl-CoA, a key component of the citric acid cycle, through a reaction catalyzed by the enzyme methylmalonyl-CoA mutase.
Structure and Function[edit | edit source]
Methylmalonyl-CoA is a derivative of coenzyme A, a molecule that is essential for many biochemical reactions in the body. The structure of methylmalonyl-CoA includes a carboxyl group, a thiol group, and a methyl group, which are attached to a ribose sugar and an adenine base.
The primary function of methylmalonyl-CoA is to facilitate the breakdown of certain amino acids and fatty acids. This process is crucial for the production of energy in the body.
Role in Metabolism[edit | edit source]
In the process of metabolism, methylmalonyl-CoA is converted into succinyl-CoA, a molecule that is a key component of the citric acid cycle. This conversion is catalyzed by the enzyme methylmalonyl-CoA mutase, which requires vitamin B12 (cobalamin) as a cofactor.
If there is a deficiency of methylmalonyl-CoA mutase or vitamin B12, the conversion of methylmalonyl-CoA to succinyl-CoA is impaired. This can lead to a buildup of methylmalonyl-CoA and related compounds in the body, a condition known as methylmalonic acidemia.
Methylmalonic Acidemia[edit | edit source]
Methylmalonic acidemia is a rare genetic disorder that affects the body's ability to break down certain proteins and fats. Symptoms can include vomiting, dehydration, weakness, and developmental delay. Treatment typically involves a low-protein diet and supplements of vitamin B12.
See Also[edit | edit source]
.svg){kind=small}
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's Wellness Encyclopedia |
| Let Food Be Thy Medicine Medicine Thy Food - Hippocrates |
Translate this page: - East Asian
中文,
日本,
한국어,
South Asian
हिन्दी,
தமிழ்,
తెలుగు,
Urdu,
ಕನ್ನಡ,
Southeast Asian
Indonesian,
Vietnamese,
Thai,
မြန်မာဘာသာ,
বাংলা
European
español,
Deutsch,
français,
Greek,
português do Brasil,
polski,
română,
русский,
Nederlands,
norsk,
svenska,
suomi,
Italian
Middle Eastern & African
عربى,
Turkish,
Persian,
Hebrew,
Afrikaans,
isiZulu,
Kiswahili,
Other
Bulgarian,
Hungarian,
Czech,
Swedish,
മലയാളം,
मराठी,
ਪੰਜਾਬੀ,
ગુજરાતી,
Portuguese,
Ukrainian
Medical Disclaimer: WikiMD is not a substitute for professional medical advice. The information on WikiMD is provided as an information resource only, may be incorrect, outdated or misleading, and is not to be used or relied on for any diagnostic or treatment purposes. Please consult your health care provider before making any healthcare decisions or for guidance about a specific medical condition. WikiMD expressly disclaims responsibility, and shall have no liability, for any damages, loss, injury, or liability whatsoever suffered as a result of your reliance on the information contained in this site. By visiting this site you agree to the foregoing terms and conditions, which may from time to time be changed or supplemented by WikiMD. If you do not agree to the foregoing terms and conditions, you should not enter or use this site. See full disclaimer.
Credits:Most images are courtesy of Wikimedia commons, and templates, categories Wikipedia, licensed under CC BY SA or similar.
Contributors: Prab R. Tumpati, MD
