ACO2
ACO2 or Aconitase 2 is an enzyme that in humans is encoded by the ACO2 gene. It is a key metabolic enzyme involved in the tricarboxylic acid cycle (TCA cycle), also known as the Krebs cycle or citric acid cycle.
Function[edit | edit source]
ACO2 is an iron-sulfur protein that contains a [4Fe-4S] cluster and catalyzes the stereo-specific isomerization of citrate to isocitrate via cis-aconitate in the second step of the TCA cycle, a key energy-yielding step in cellular metabolism.
Clinical significance[edit | edit source]
Mutations in the ACO2 gene are associated with Infantile cerebellar-retinal degeneration and Optic atrophy 9. These conditions are characterized by progressive loss of vision and coordination, as well as a range of other neurological symptoms.
Structure[edit | edit source]
The ACO2 protein is a homodimer, with each subunit containing a [4Fe-4S] cluster. The protein is located in the mitochondria, where it plays a crucial role in energy production.
See also[edit | edit source]
References[edit | edit source]
External links[edit | edit source]
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.
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
Contributors: Prab R. Tumpati, MD