Methylmalonyl-CoA
Methylmalonyl-CoA[edit | edit source]
Methylmalonyl-CoA is an important intermediate in the metabolism of certain amino acids, odd-chain fatty acids, and cholesterol. It plays a crucial role in the conversion of propionyl-CoA to succinyl-CoA, a process that is essential for the entry of carbon atoms into the citric acid cycle.
Biochemical Pathway[edit | edit source]
Methylmalonyl-CoA is formed from propionyl-CoA through a carboxylation reaction catalyzed by the enzyme propionyl-CoA carboxylase. This reaction requires biotin as a cofactor and results in the formation of D-methylmalonyl-CoA. The D-methylmalonyl-CoA is then converted to L-methylmalonyl-CoA by the enzyme methylmalonyl-CoA epimerase.
The next step involves the conversion of L-methylmalonyl-CoA to succinyl-CoA by the enzyme methylmalonyl-CoA mutase. This reaction requires adenosylcobalamin, a form of vitamin B12, as a cofactor. Succinyl-CoA can then enter the citric acid cycle, where it is further metabolized to produce energy.
Clinical Significance[edit | edit source]
Deficiencies in the enzymes involved in the conversion of methylmalonyl-CoA to succinyl-CoA can lead to metabolic disorders such as methylmalonic acidemia. This condition is characterized by the accumulation of methylmalonic acid in the blood, which can lead to metabolic acidosis and other serious health issues.
Vitamin B12 deficiency can also result in elevated levels of methylmalonyl-CoA, as the conversion to succinyl-CoA is impaired. This is often used as a diagnostic marker for vitamin B12 deficiency.
Related Pages[edit | edit source]
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