sphingolipids

Glucocerebrosides is a natural product.

3-Hydroxybutyric acid (Butanoic acid) (or beta-hydroxybutyrate) is a ketone body. Like the other ketone bodies (acetoacetate and acetone), levels of 3-hydroxybutyrate in blood and urine are raised in ketosis. In humans, 3-hydroxybutyrate is synthesized in the liver from acetyl-CoA and can be used as an energy source by the brain when blood glucose is low. Blood levels of 3-hydroxybutyric acid levels may be monitored in diabetic patients to look for diabetic ketoacidosis. Persistent mild hyperketonemia is a common finding in newborns. Ketone bodies serve as an indispensable source of energy for extrahepatic tissues, especially the brain and lung of developing mammals. Another important function of ketone bodies is to provide acetoacetyl-CoA and acetyl-CoA for the synthesis of cholesterol, fatty acids, and complex lipids. During the early postnatal period, acetoacetate (AcAc) and beta-hydroxybutyrate are preferred over glucose as substrates for synthesis of phospholipids and sphingolipids in accord with requirements for brain growth and myelination. Thus, during the first 2 weeks of postnatal development, when the accumulation of cholesterol and phospholipids accelerates, the proportion of ketone bodies incorporated into these lipids increases. On the other hand, an increased proportion of ketone bodies is utilized for cerebroside synthesis during the period of active myelination. In the lung, AcAc serves better than glucose as a precursor for the synthesis of lung phospholipids. The synthesized lipids, particularly dipalmitoylphosphatidylcholine, are incorporated into surfactant, and thus have a potential role in supplying adequate surfactant lipids to maintain lung function during the early days of life (PMID: 3884391 ). 3-Hydroxybutyric acid is found to be associated with fumarase deficiency and medium-chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism.

Pyridoxine hydrochloride (Vitamin B6) is the 4-methanol form of vitaminB6 which is converted to pyridoxal phosphate which is a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid.

Nervonic acid (Selacholeic acid) is a long chain unsaturated fatty acid that is enriched in sphingomyelin. It consists of choline, sphingosine, phosphoric acid, and fatty acid. Nervonic acid may enhance the brain functions and prevent demyelination (Chemical Land21). Research shows that there is negative relationship between nervonic acid and obesity-related risk factors. Demyelination in adrenoleukodystrophy (ALD) is associated with an accumulation of very long chain saturated fatty acids stemming from a genetic defect in the peroxisomal beta oxidation system responsible for the chain shortening of these fatty acids. Sphingolipids from post mortem ALD brain have decreased levels of nervonic acid, 24:1(n-9), and increased levels of stearic acid, 18:0.

Fumonisin B1 (Fumonisin-B1) is a mycotoxin produced by Fusarium oxysporum that is widely present in crops, contaminating them and posing a significant food safety hazard.Fumonisin B1 is structurally a sphingosine and sphingomyelin analog, and inhibits the biosynthesis of complex sphingolipids through the inhibition of ceramidic synthases.

Ganglioside GM1 is one of the major sphingolipids (GSLs) on the cell surface of the central nervous system (CNS), which is protective of the CNS and is used in the study of neurological disorders.Ganglioside GM1 exerts protection against glutamate excitotoxicity through its oligosaccharides in wild-type and amyotrophic lateral sclerosis motor neurons.

The 4-carboxyaldehyde form of vitamin B6 which is converted to pyridoxal phosphate which is a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid.

Sphingosine (d17:1) (C17 Sphingosine) is a 17-carbon sphingolipid found in human skin that can be phosphorylated by sphingomyelin kinase.Sphingosine C-17 can be used as an internal standard to perform spectroscopic analysis of sphingolipids.