Page last updated: 2024-10-24

carnitine biosynthetic process

Definition

Target type: biologicalprocess

The chemical reactions and pathways resulting in the formation of carnitine (hydroxy-trimethyl aminobutyric acid), a compound that participates in the transfer of acyl groups across the inner mitochondrial membrane. [GOC:jl, ISBN:0198506732]

Carnitine biosynthesis is a complex process that involves multiple steps and enzymes. It begins with the amino acid lysine, which is converted to trimethyllysine by a series of enzymatic reactions. Trimethyllysine is then hydroxylated to produce 6-N-trimethyllysine. This hydroxylated form undergoes a series of enzymatic transformations, including a decarboxylation reaction, to produce γ-butyrobetaine. The final step in carnitine biosynthesis is the oxidation of γ-butyrobetaine to carnitine by the enzyme γ-butyrobetaine dioxygenase. Carnitine is a vital nutrient for the body, playing a crucial role in the transport of long-chain fatty acids into mitochondria for energy production. It also plays a role in the regulation of blood sugar levels and the removal of waste products from the body.'
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Proteins (3)

ProteinDefinitionTaxonomy
Serine hydroxymethyltransferase, cytosolicA serine hydroxymethyltransferase, cytosolic that is encoded in the genome of human. [PRO:DNx, UniProtKB:P34896]Homo sapiens (human)
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 (9)

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
pemetrexedpemetrexed disodium : An organic sodium salt that is the disodium salt of N-{4-[2-(2-amino-4-oxo-4,7-dihydro-1H-pyrrolo[2,3-d]pyrimidin-5-yl)ethyl]benzoyl}-L-glutamic acid. Inhibits thymidylate synthase (TS), 421 dihydrofolate reductase (DHFR), and glycinamide ribonucleotide formyltransferase (GARFT).N-acyl-L-glutamic acid;
pyrrolopyrimidine
antimetabolite;
antineoplastic agent;
EC 1.5.1.3 (dihydrofolate reductase) inhibitor;
EC 2.1.1.45 (thymidylate synthase) inhibitor;
EC 2.1.2.2 (phosphoribosylglycinamide formyltransferase) inhibitor