Page last updated: 2024-10-24

gamma-butyrobetaine dioxygenase activity

Definition

Target type: molecularfunction

Catalysis of the reaction: 2-oxoglutarate + 4-(trimethylammonio)butanoate + O2 = carnitine + CO2 + succinate. [EC:1.14.11.1, RHEA:24028]

Gamma-butyrobetaine dioxygenase (GBSD) is an enzyme responsible for the crucial step in the biosynthesis of L-carnitine, a vital nutrient involved in fatty acid metabolism. Specifically, GBSD catalyzes the oxygen-dependent oxidation of gamma-butyrobetaine, a precursor to carnitine, into 4-hydroxybutyrobetaine. This oxidative process involves the incorporation of molecular oxygen into the substrate, resulting in the formation of a hydroxyl group at the 4-position of the butyrobetaine molecule. The reaction occurs in a two-step mechanism. First, GBSD binds both gamma-butyrobetaine and oxygen, forming a complex. Then, GBSD facilitates the transfer of an oxygen atom from molecular oxygen to gamma-butyrobetaine, leading to the production of 4-hydroxybutyrobetaine and water. This enzymatic reaction requires the presence of iron ions, which act as cofactors in the active site of GBSD. The iron ions facilitate the transfer of electrons during the oxidation process. GBSD activity plays a pivotal role in carnitine biosynthesis, which is essential for the transport of long-chain fatty acids across mitochondrial membranes. This process is crucial for energy production, as fatty acids serve as an important source of fuel in many tissues. Furthermore, carnitine is involved in other cellular processes, such as muscle function and detoxification. Deficiencies in GBSD activity can lead to carnitine deficiency, which can manifest in a range of clinical symptoms, including muscle weakness, fatigue, and impaired cognitive function. Therefore, understanding the molecular function of GBSD is essential for comprehending the role of carnitine in human health and disease.'
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Proteins (2)

ProteinDefinitionTaxonomy
Gamma-butyrobetaine dioxygenaseA gamma-butyrobetaine dioxygenase that is encoded in the genome of human. [PRO:DNx, UniProtKB:O75936]Homo sapiens (human)
Gamma-butyrobetaine dioxygenaseA gamma-butyrobetaine dioxygenase that is encoded in the genome of human. [PRO:DNx, UniProtKB:O75936]Homo sapiens (human)

Compounds (8)

CompoundDefinitionClassesRoles
ebselenebselen : A benzoselenazole that is 1,2-benzoselenazol-3-one carrying an additional phenyl substituent at position 2. Acts as a mimic of glutathione peroxidase.benzoselenazoleanti-inflammatory drug;
antibacterial agent;
anticoronaviral agent;
antifungal agent;
antineoplastic agent;
antioxidant;
apoptosis inducer;
EC 1.13.11.33 (arachidonate 15-lipoxygenase) inhibitor;
EC 1.13.11.34 (arachidonate 5-lipoxygenase) inhibitor;
EC 1.3.1.8 [acyl-CoA dehydrogenase (NADP(+))] inhibitor;
EC 1.8.1.12 (trypanothione-disulfide reductase) inhibitor;
EC 2.5.1.7 (UDP-N-acetylglucosamine 1-carboxyvinyltransferase) inhibitor;
EC 2.7.10.1 (receptor protein-tyrosine kinase) inhibitor;
EC 3.1.3.25 (inositol-phosphate phosphatase) inhibitor;
EC 3.4.22.69 (SARS coronavirus main proteinase) inhibitor;
EC 3.5.4.1 (cytosine deaminase) inhibitor;
EC 5.1.3.2 (UDP-glucose 4-epimerase) inhibitor;
enzyme mimic;
ferroptosis inhibitor;
genotoxin;
hepatoprotective agent;
neuroprotective agent;
radical scavenger
succinylacetone4,6-dioxoheptanoic acid : A dioxo monocarboxylic acid that is heptanoic acid in which oxo groups replace the hydrogens at positions 4 and 6. It is an abnormal metabolite of the tyrosine metabolic pathway and a marker for type 1 tyrosinaemia.

succinylacetone: inhibitor of heme biosynthesis
beta-diketone;
dioxo monocarboxylic acid
human metabolite
thiramthiram : An organic disulfide that results from the formal oxidative dimerisation of N,N-dimethyldithiocarbamic acid. It is widely used as a fungicidal seed treatment.

Thiram: A dithiocarbamate chemical, used commercially in the rubber processing industry and as a fungicide. In vivo studies indicate that it inactivates the enzyme GLUTATHIONE REDUCTASE. It has mutagenic activity and may induce chromosomal aberrations.
organic disulfideantibacterial drug;
antifungal agrochemical;
antiseptic drug
daminozidedaminozide: induces tumorsstraight-chain fatty acid
diphenyldiselenidediphenyldiselenide: structure given in first source
benzeneseleninic acidbenzeneseleninic acid: structure given in first source
3-(2,2,2-trimethylhydrazine)propionate3-(2,2,2-trimethylhydrazine)propionate: structural analog of gamma-butyrobetaine, also of carnitine; antianginal compound; MET-88 is dihydrate; structure given in first source

meldonium : An ammonium betaine that is beta-alaninate in which one of the amino hydrogens is replaced by a trimethylamino group. A clinically used cardioprotective drug that is used for treatment of heart failure, myocardial infarction, arrhythmia, atherosclerosis and diabetes.
ammonium betainecardioprotective agent;
EC 1.14.11.1 (gamma-butyrobetaine dioxygenase) inhibitor;
neuroprotective agent
crotonic acid betainecrotonic acid betaine: RN given refers to inner salt without isomeric designation4-(trimethylammonio)but-2-enoate