fatty acid metabolism

Coenzyme A is an obligatory cofactor in all living cells synthesized from pantothenate (Vitamin B5), adenosine triphosphate (ATP), and cysteine.

L-Carnitine (L(-)-Carnitine) is an amino acid derivative. L-Carnitine facilitates long-chain fatty acid entry into mitochondria, delivering substrate for oxidation and subsequent energy production. Fatty acids are utilized as an energy substrate in all tissues except the brain.

Thiamine hydrochloride (Vitamin B1) is an essential micronutrient and a cofactor for many central metabolic enzymes.

Biotin (Vitamin H) is a water-soluble B-vitamin and is necessary for cell growth, the production of fatty acids, and the metabolism of fats and amino acids.

2,2-Dihydroxyacetic acid (Formylformic acid) is an intermediate in the glyoxalate cycle, which enables certain organisms to convert fatty acids to carbohydrates.2,2-Dihydroxyacetic acid has been found to be associated with primary hyperoxaluria, an inborn error of metabolism. As an aldehyde, glyoxalate is also highly active and can modify proteins to form advanced glycosylation products (AGEs).

Increased levels of erucic acid (22:1n9) have been found in the red blood cell membranes of autistic subjects with developmental regression (PMID: 16581239 ). Erucic acid (13(Z)-Docosenoic Acid) is broken down long-chain acyl-coenzyme A (CoA) dehydrogenase, which is produced in the liver. This enzyme breaks this long chain fatty acid into shorter-chain fatty acids. human infants have relatively low amounts of this enzyme and because of this, babies should not be given foods high in erucic acid. Erucic acid is found to be associated with isovaleric acidemia, which is an inborn error of metabolism.

Butanoic acid is an endogenous human metabolite, an endogenous histone deacetylase (HDAC) inhibitor. The IC50 of HDAC3, HDAC4 and HDAC1 were 2.4 mM, 4.5 mM and 5.3 mM, respectively. 3-Hydroxybutyric acid has the effects of energy metabolism, neuroprotection, anti-inflammatory, and improvement of insulin resistance.

Trans-2-butene-1,4-dicarboxylic acid (3-Hexenedioic Acid) is a normal human unsaturated dicarboxylic acid metabolite with increased excretion in patients with Dicarboxylic aciduria caused by fatty acid metabolism disorders The urinary excretion of Trans-2-butene-1,4-dicarboxylic acid is increased in conditions of augmented mobilization of fatty acids or inhibited fatty acid oxidation.

D-Pantothenic acid (vitamin B5) is a water-soluble vitamin ubiquitously found in plants and animal tissues with an antioxidant property. D-Pantothenic acid is a component of coenzyme A (CoA) and a part of the vitamin B2 complex. D-Pantothenic acid is a growth factor and is essential for various metabolic functions, including the metabolism of carbohydrates, proteins, and fatty acids. This vitamin is also involved in the synthesis of cholesterol, lipids, neurotransmitters, steroid hormones, and hemoglobin.

12-oxo Leukotriene B4 (12-Oxo-LTB4) is a potent pro-inflammatory lipid mediator classified as a dihydroxy fatty acid that is enzymatically derived from arachidonic acid metabolism via the 5-lipoxygenase (5-LO) pathway, and it promotes a diverse array of leukocyte functional responses including cellular aggregation, stimulation of transmembrane ion fluxes, enhancement of lysosomal enzyme release, superoxide anion generation, chemotaxis, and chemokinesis; 12-oxo Leukotriene B4 is an initial oxidative metabolite formed from Leukotriene B4 via the LTB4 12-hydroxydehydrogenase pathway, which undergoes rapid enzymatic conversion first to 10,11-dihydro-12-oxo-LTB4 followed by reduction of the 12-oxo group to yield 10,11-dihydro-LTB4, and this metabolite exhibits significantly reduced biological potency, being approximately 70-fold less potent than LTB4 in stimulating Ca2+ mobilization in human neutrophils (EC50 = 33 nM vs. 0.46 nM) and also markedly less effective at stimulating neutrophil migration (EC50 = 170 nM vs. 2.7 nM).

Taurohyodeoxycholic acid (THDCA) is a bile acid that plays a key role in regulating the hepatic inflammatory microenvironment.THDCA reduces the expression of TNF-α and IL-6 and inhibits the development of hepatocellular carcinoma (HCC). It is able to inhibit non-alcoholic fatty liver disease and regulate bile acid metabolism in combination with astragalus polysaccharides: it reduces CD36 expression in the liver and decreases hepatic lipid accumulation by up-regulating CYP7B1 and down-regulating CYP7A1.

15-methyl Palmitic Acid is a methylated fatty acid that is widely found in animal lipids and may be involved in the regulation of fat metabolism.

Acetoacetic acid lithium salt (Lithium 3-Oxobutyrate) is a weak organic acid that can be produced in the human liver under certain conditions of poor Metabolism leading to excessive fatty acid breakdown (diabetes mellitus leading to diabetic ketoacidosis).

N-(3-Phenylpropionyl)glycine (Phenylpropionylglycine) is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. Acyl glycines are produced through the action of glycine N-acyltransferase which is an enzyme that catalyzes the chemical reaction:acyl-CoA + glycine < - > CoA + N-acylglycineThe detection of phenylpropionylglycine in urine after an oral load of phenylpropionic acid can be used to diagnose deficiency of medium-chain acyl-CoA dehydrogenase, a frequent and treatable metabolic defect.

N-Isovaleroylglycine (Isovaleroylglycine) is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases, the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase which is an enzyme that catalyzes the chemical reaction: acyl-CoA + glycine < -- > CoA + N-acylglycine. N-Isovaleroylglycine is a byproduct of the catabolism of the amino acid leucine. Accumulation of N-Isovaleroylglycine occurs in Isovaleric Acidemia (IVA).

4-Oxododecanedioic acid is an organic acid containing a ketone group that can be used in studies related to organic synthesis and fatty acid metabolism. Studies related to its structural analog dodecanedioic acid have found that intake of long-chain dicarboxylic acid increases beta-oxidation of fatty acids, decreases body fat accumulation, and improves glucose tolerance.

Biotin (Standard) is the standard substance of Biotin, and it is applicable for quantitative analysis, quality control, and related research in biochemical experiments. Biotin (Vitamin H) is a naturally occurring, water-soluble B vitamin involved in fatty acid production, fat and amino acid metabolism, and cell growth. Biotin is often used for biotin labeling.

Erucic acid (Standard) is the standard substance of Erucic acid, and it is applicable for quantitative analysis, quality control, and related research in biochemical experiments. Increased levels of erucic acid (22:1n9) have been found in the red blood cell membranes of autistic subjects with developmental regression (PMID: 16581239 ). Erucic acid (13(Z)-Docosenoic Acid) is broken down long-chain acyl-coenzyme A (CoA) dehydrogenase, which is produced in the liver. This enzyme breaks this long chain fatty acid into shorter-chain fatty acids. human infants have relatively low amounts of this enzyme and because of this, babies should not be given foods high in erucic acid. Erucic acid is found to be associated with isovaleric acidemia, which is an inborn error of metabolism.

Docosahexaenoyl-L-carnitine chloride is a long-chain acyl-L-carnitine composed of Docosahexaenoic acid and L-carnitine. Under the influence of carnitine palmitoyltransferase I (CPT I) and other enzymes, it is transported to the mitochondria for β-oxidation and breakdown. Docosahexaenoyl-L-carnitine chloride holds potential for research in diseases related to fatty acid metabolism.