3-hydroxyisobutyrate dehydrogenase
| 3-hydroxyisobutyrate dehydrogenase | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
| EC number | 1.1.1.31 | ||||||||
| CAS number | 9028-54-0 | ||||||||
| Databases | |||||||||
| IntEnz | IntEnz view | ||||||||
| BRENDA | BRENDA entry | ||||||||
| ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| Gene Ontology | AmiGO / QuickGO | ||||||||
| |||||||||
3-Hydroxyisobutyrate dehydrogenase is an enzyme that plays a crucial role in the metabolism of the amino acid valine. It catalyzes the reversible oxidation of 3-hydroxyisobutyrate to methylmalonate semialdehyde, using NAD+ as a cofactor, which is reduced to NADH in the process.
Function[edit | edit source]
3-Hydroxyisobutyrate dehydrogenase is involved in the catabolic pathway of valine, an essential amino acid. This pathway is important for the production of energy and the generation of intermediates that can enter the citric acid cycle. The enzyme's activity is crucial for maintaining the balance of amino acid levels and for the proper functioning of metabolic processes.
Structure[edit | edit source]
The enzyme is a member of the oxidoreductase family, specifically those acting on the CH-OH group of donors with NAD+ or NADP+ as acceptor. The structure of 3-hydroxyisobutyrate dehydrogenase has been studied in various organisms, revealing a conserved domain that is responsible for its catalytic activity.
Clinical significance[edit | edit source]
Deficiencies or malfunctions in 3-hydroxyisobutyrate dehydrogenase can lead to metabolic disorders. Since it is involved in valine metabolism, any disruption can result in the accumulation of toxic intermediates, potentially leading to conditions such as maple syrup urine disease or other metabolic syndromes.
Research[edit | edit source]
Ongoing research is focused on understanding the detailed mechanism of action of 3-hydroxyisobutyrate dehydrogenase, its regulation, and its role in various metabolic pathways. Studies are also exploring its potential as a target for therapeutic interventions in metabolic disorders.
Also see[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 Wikipedia, licensed under CC BY SA or similar.
Contributors: Prab R. Tumpati, MD
