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)
| Protein | Definition | Taxonomy |
|---|---|---|
| Serine hydroxymethyltransferase, cytosolic | A serine hydroxymethyltransferase, cytosolic that is encoded in the genome of human. [PRO:DNx, UniProtKB:P34896] | Homo sapiens (human) |
| Gamma-butyrobetaine dioxygenase | A gamma-butyrobetaine dioxygenase that is encoded in the genome of human. [PRO:DNx, UniProtKB:O75936] | Homo sapiens (human) |
| Gamma-butyrobetaine dioxygenase | A gamma-butyrobetaine dioxygenase that is encoded in the genome of human. [PRO:DNx, UniProtKB:O75936] | Homo sapiens (human) |
Compounds (9)
| Compound | Definition | Classes | Roles |
|---|---|---|---|
| ebselen | ebselen : A benzoselenazole that is 1,2-benzoselenazol-3-one carrying an additional phenyl substituent at position 2. Acts as a mimic of glutathione peroxidase. | benzoselenazole | anti-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 |
| succinylacetone | 4,6-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 |
| thiram | thiram : 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 disulfide | antibacterial drug; antifungal agrochemical; antiseptic drug |
| daminozide | daminozide: induces tumors | straight-chain fatty acid | |
| diphenyldiselenide | diphenyldiselenide: structure given in first source | ||
| benzeneseleninic acid | benzeneseleninic acid: structure given in first source | ||
| 3-(2,2,2-trimethylhydrazine)propionate | 3-(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 betaine | cardioprotective agent; EC 1.14.11.1 (gamma-butyrobetaine dioxygenase) inhibitor; neuroprotective agent |
| crotonic acid betaine | crotonic acid betaine: RN given refers to inner salt without isomeric designation | 4-(trimethylammonio)but-2-enoate | |
| pemetrexed | pemetrexed 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 |