Beta-ketothiolase
Beta-ketothiolase (also known as Acetoacetyl-CoA thiolase) is an enzyme that plays a significant role in the metabolism of fatty acids and amino acids. It is involved in the ketone body and isoleucine catabolic pathways.
Function[edit | edit source]
Beta-ketothiolase specifically catalyzes the third step of ketone body degradation, which is the CoA-mediated thiolytic cleavage of acetoacetyl-CoA to yield 2 molecules of acetyl-CoA. In addition, it is also involved in the degradation of isoleucine and the anti catabolism of 2-methylacetoacetyl-CoA.
Clinical significance[edit | edit source]
Deficiency of this enzyme, an autosomal recessive inherited disorder, results in beta-ketothiolase deficiency, a metabolic disorder that can lead to metabolic acidosis, ketosis, and episodic encephalopathy.
See also[edit | edit source]
References[edit | edit source]
External links[edit | edit source]
| Beta-ketothiolase Resources | |
|---|---|
|
 |
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
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
