A subclass of GLYCOSPHINGOLIPIDS containing large polar heads made up of several sugar units. One or more of their terminal sugar units are bound to a negatively charged molecule at pH 7. Members of this class include: GANGLIOSIDES, uronoglycosphingolipids, SULFOGLYCOSPHINGOLIPIDS, phosphoglycosphingolipids, and phosphonoglycosphingolipids.
Lipids containing at least one monosaccharide residue and either a sphingoid or a ceramide (CERAMIDES). They are subdivided into NEUTRAL GLYCOSPHINGOLIPIDS comprising monoglycosyl- and oligoglycosylsphingoids and monoglycosyl- and oligoglycosylceramides; and ACIDIC GLYCOSPHINGOLIPIDS which comprises sialosylglycosylsphingolipids (GANGLIOSIDES); SULFOGLYCOSPHINGOLIPIDS (formerly known as sulfatides), glycuronoglycosphingolipids, and phospho- and phosphonoglycosphingolipids. (From IUPAC's webpage)
GLYCOSPHINGOLIPIDS with a sulfate group esterified to one of the sugar groups.
Chromatography on thin layers of adsorbents rather than in columns. The adsorbent can be alumina, silica gel, silicates, charcoals, or cellulose. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
The sequence of carbohydrates within POLYSACCHARIDES; GLYCOPROTEINS; and GLYCOLIPIDS.
A subclass of GLYCOSPHINGOLIPIDS containing one or more sugars within their head group connected directly to a ceramide moiety. They consist of monoglycosyl-, and oligoglycosylsphingoids and monoglycosyl- and oligoglycosylceramides.
Glycosphingolipids which contain as their polar head group a lactose moiety bound in glycosidic linkage to the hydroxyl group of ceramide. Their accumulation in tissue, due to a defect in lactosylceramide beta-galactosidase, is the cause of lactosylceramidosis.
A subclass of ACIDIC GLYCOSPHINGOLIPIDS. They contain one or more sialic acid (N-ACETYLNEURAMINIC ACID) residues. Using the Svennerholm system of abbrevations, gangliosides are designated G for ganglioside, plus subscript M, D, or T for mono-, di-, or trisialo, respectively, the subscript letter being followed by a subscript arabic numeral to indicated sequence of migration in thin-layer chromatograms. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1997)
Glycosphingolipids containing N-acetylglucosamine (paragloboside) or N-acetylgalactosamine (globoside). Globoside is the P antigen on erythrocytes and paragloboside is an intermediate in the biosynthesis of erythrocyte blood group ABH and P 1 glycosphingolipid antigens. The accumulation of globoside in tissue, due to a defect in hexosaminidases A and B, is the cause of Sandhoff disease.
Cerebrosides which contain as their polar head group a glucose moiety bound in glycosidic linkage to the hydroxyl group of ceramides. Their accumulation in tissue, due to a defect in beta-glucosidase, is the cause of Gaucher's disease.
Neutral glycosphingolipids that contain a monosaccharide, normally glucose or galactose, in 1-ortho-beta-glycosidic linkage with the primary alcohol of an N-acyl sphingoid (ceramide). In plants the monosaccharide is normally glucose and the sphingoid usually phytosphingosine. In animals, the monosaccharide is usually galactose, though this may vary with the tissue and the sphingoid is usually sphingosine or dihydrosphingosine. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1st ed)
Any compound containing one or more monosaccharide residues bound by a glycosidic linkage to a hydrophobic moiety such as an acylglycerol (see GLYCERIDES), a sphingoid, a ceramide (CERAMIDES) (N-acylsphingoid) or a prenyl phosphate. (From IUPAC's webpage)
A ganglioside present in abnormally large amounts in the brain and liver due to a deficient biosynthetic enzyme, G(M3):UDP-N-acetylgalactosaminyltransferase. Deficiency of this enzyme prevents the formation of G(M2) ganglioside from G(M3) ganglioside and is the cause of an anabolic sphingolipidosis.
Members of the class of neutral glycosphingolipids. They are the basic units of SPHINGOLIPIDS. They are sphingoids attached via their amino groups to a long chain fatty acyl group. They abnormally accumulate in FABRY DISEASE.
A class of membrane lipids that have a polar head and two nonpolar tails. They are composed of one molecule of the long-chain amino alcohol sphingosine (4-sphingenine) or one of its derivatives, one molecule of a long-chain acid, a polar head alcohol and sometimes phosphoric acid in diester linkage at the polar head group. (Lehninger et al, Principles of Biochemistry, 2nd ed)

Monocytic cell activation by Nonendotoxic glycoprotein from Prevotella intermedia ATCC 25611 is mediated by toll-like receptor 2. (1/12)

Lipopolysaccharide (LPS) preparations from gram-negative black-pigmented bacteria such as Porphyromonas gingivalis and Prevotella intermedia activate cells from non-LPS-responsive C3H/HeJ mice, but it is still unclear whether this activity is due to the unique structure of LPS or to a minor component(s) responsible for the activity in the preparation. A nonendotoxic glycoprotein with bioactivity against cells from C3H/HeJ mice was purified from a hot phenol-water extract of P. intermedia ATCC 25611 and designated Prevotella glycoprotein (PGP). Treatment of human monocytic THP-1 cells with 22-oxyacalcitriol (OCT) induced maturation and marked expression of CD14 on the cells, but the cells constitutively expressed Toll-like receptor 2 (TLR2) and TLR4 on the cells irrespective of the treatment. PGP induced a high level of interleukin-8 production at doses of 100 ng/ml and higher in OCT-treated THP-1 cells compared with Salmonella LPS, and the production was significantly inhibited by anti-CD14 and anti-TLR2 but not anti-TLR4 antibodies. Consistent with this, TLR2-deficient murine macrophages did not respond to PGP. It was also shown that PGP activity on the THP-1 cells was LPS-binding protein dependent and was inhibited by a synthetic lipid A precursor IV(A). These results indicate that PGP activates monocytic cells in a CD14- and TLR2-dependent manner.  (+info)

Structural elucidation of novel phosphocholine-containing glycosylinositol-phosphoceramides in filamentous fungi and their induction of cell death of cultured rice cells. (2/12)

Novel ZGLs (zwitterionic glycosphingolipids) have been found in and extracted from the mycelia of filamentous fungi ( Acremonium sp.) isolated from soil. Five ZGLs (ZGL1-ZGL5) were structurally elucidated by sugar compositional analysis, methylation analysis, periodate oxidation, matrix-assisted laser-desorption ionization-time-of-flight MS, (1)H-NMR spectroscopy and fast-atom bombardment MS. Their chemical structures were as follows: GlcN(alpha1-2)Ins1-P-1Cer (ZGL1), Man(alpha1-6)GlcN(alpha1-2)Ins1-P-1Cer (ZGL2), Man(alpha1-6)Man(alpha1-6)GlcN(alpha1-2)Ins1-P-1Cer (ZGL3), PC-->6Man(alpha1-6)GlcN(alpha1-2)Ins1- P -1Cer (ZGL4), and PC-->6Man(alpha1-6)Man(alpha1-6)GlcN(alpha1-2)Ins1-P-1Cer (ZGL5) (where Cer is ceramide and PC is phosphocholine). In addition, one acidic glycosphingolipid, which was the precursor of ZGLs, was also characterized as inositol-phosphoceramide. The core structure of the ZGLs, GlcN(alpha1-2)Ins1- P, is rather different from those found in other fungi, such as Man(alpha1-2)Ins1- P and Man(alpha1-6)Ins1- P. Interestingly, the terminal mannose residue of ZGL4 and ZGL5 was modified further with a PC group. The presence of PC-containing glycosylinositol-phosphoceramides has not been reported previously in any organism. The ceramide constituents of both ZGLs and acidic glycosphingolipid were essentially the same, and consisted of a 4-hydroxyoctadecasphinganine (phytosphingosine) as the sole sphingoid base and 2-hydroxytetracosanoic acid (>90%) as the major fatty acid. ZGLs were found to cause cell death in suspensions of cultured rice cells. The cell death-inducing activity of ZGLs is probably due to the characteristic glycan moiety of Man(alpha1-6)GlcN, and PC-containing ZGLs had high activity. This study is the first to demonstrate that fungal glycosylinositol-phosphoceramides induce cell death in cultured rice cells.  (+info)

Role of multiple drug resistance protein 1 in neutral but not acidic glycosphingolipid biosynthesis. (3/12)

Transfection studies have implicated the multiple drug resistance pump, MDR1, as a glucosyl ceramide translocase within the Golgi complex (Lala, P., Ito, S., and Lingwood, C. A. (2000) J. Biol. Chem. 275, 6246-6251). We now show that MDR1 inhibitors, cyclosporin A or ketoconazole, inhibit neutral glycosphingolipid biosynthesis in 11 of 12 cell lines tested. The exception, HeLa cells, do not express MDR1. Microsomal lactosyl ceramide and globotriaosyl ceramide synthesis from endogenous or exogenously added liposomal glucosyl ceramide was inhibited by cyclosporin A, consistent with a direct role for MDR1/glucosyl ceramide translocase activity in their synthesis. In contrast, cellular ganglioside synthesis in the same cells, was unaffected by MDR1 inhibition, suggesting neutral and acid glycosphingolipids are synthesized from distinct precursor glycosphingolipid pools. Metabolic labeling in wild type and knock-out (MDR1a, 1b, MRP1) mouse fibroblasts showed the same loss of neutral glycosphingolipid (glucosyl ceramide, lactosyl ceramide) but not ganglioside (GM3) synthesis, confirming the proposed role for MDR1 translocase activity. Cryo-immunoelectron microscopy showed MDR1 was predominantly intracellular, largely in rab6-containing Golgi vesicles and Golgi cisternae, the site of glycosphingolipid synthesis. These studies identify MDR1 as the major glucosyl ceramide flippase required for neutral glycosphingolipid anabolism and demonstrate a previously unappreciated dichotomy between neutral and acid glycosphingolipid synthesis.  (+info)

Characterization of two novel pyruvylated glycosphingolipids containing 2'-aminoethylphosphoryl(-->6)-galactose from the nervous system of Aplysia kurodai. (4/12)

Two novel acidic glycosphingolipids containing pyruvylated galactose were purified from the nervous tissue of Aplysia kurodai by successive Iatrobeads column chromatographies. By component analysis, sugar analysis, permethylation studies, fast atom bombardment-mass spectrometry, and proton magnetic resonance spectrometry, the structures of these acidic glycosphingolipids, named F-9 and FGL-I, were determined to be: [3,4-O-(S-1-carboxyethylidene)]Gal beta 1-->3 GalNAc alpha 1-->3[6'-O-(2-aminoethylphosphonyl)Gal alpha 1-->2] (2-aminoethylphosphoryl 1-->6)Gal beta 1-->4Glc beta 1-->1ceramide and [3,4-O-(S-1-carboxyethylidene)] Gal beta 1-->3GalNAc alpha 1-->3(Fuc alpha 1-->2)(2-aminoethylphosphonyl-->6 Gal beta 1-->4Glc beta 1-->1ceramide, octadeca-4-sphingenine and anteisononadeca-4-sphingenine. Thus, pyruvylated glycosphingolipids containing phosphoethanolamine in addition to or in place of 2-aminoethylphosphonate are present in the nervous system of Aplysia.  (+info)

Characterization of neutral and acidic glycosphingolipids from the lectin-producing mushroom, Polyporus squamosus. (5/12)

The polypore mushroom Polyporus squamosus is the source of a lectin that exhibits a general affinity for terminal beta-galactosides, but appears to have an extended carbohydrate-binding site with high affinity and strict specificity for the nonreducing terminal trisaccharide sequence NeuAcalpha2 --> 6Galbeta1 --> 4Glc/GlcNAc. In considering the possibility that the lectin's in vivo function could involve interaction with an endogenous glycoconjugate, it would clearly be helpful to identify candidate ligands among various classes of carbohydrate-containing materials expressed by P. squamosus. Since evidence has been accumulating that glycosphingolipids (GSLs) may serve as key ligands for some endogenous lectins in animal species, possible similar roles for fungal GSLs could be considered. For this study, total lipids were extracted from mature fruiting body of P. squamosus. Multistep fractionation yielded a major monohexosylceramide (CMH) component and three major glycosylinositol phosphorylceramides (GIPCs) from the neutral and acidic lipids, respectively. These were characterized by a variety of techniques as required, including one- and two-dimensional (1)H- and (13)C-nuclear magnetic resonance (NMR) spectroscopy; electrospray ionization-mass spectrometry (ESI-MS, tandem-MS/collision-induced decay-MS, and ion trap-MS(n)); and component and methylation linkage analysis by gas chromatography-mass spectrometry. The CMH was determined to be glucosylceramide having a typical ceramide consisting of 2-hydroxy fatty-N-acylated (4E,8E)-9-methyl-sphinga-4,8-dienine. The GIPCs were identified as Manalpha1 --> 2Ins1-P-1Cer (Ps-1), Galbeta1 --> 6Manalpha1 --> 2Ins1-P-1Cer (Ps-2), and Manalpha1 --> 3Fucalpha1 --> 2Galalpha1 --> 6Galbeta1 --> 6Manalpha1 -->2Ins1-P-1Cer (Ps-5), respectively (where Ins = myo-inositol, P = phosphodiester, and Cer = ceramide consisting mainly of long-chain 2-hydroxy and 2,3-dihydroxy fatty-N-acylated 4-hydroxy-sphinganines). Of these GSLs, Ps-2 could potentially interact with P. squamosus lectin, and further investigations will focus on determining the binding affinity, if any, of the lectin for the GIPCs isolated from this fungus.  (+info)

Activation of invariant NKT cells by toll-like receptor 9-stimulated dendritic cells requires type I interferon and charged glycosphingolipids. (6/12)

Invariant natural killer T (iNKT) cells are a subset of innate lymphocytes that recognize lipid antigens in the context of CD1d and mediate potent immune regulatory functions via the rapid production of interferon-gamma (IFN-gamma) and interleukin-4 (IL-4). We investigated whether diverse Toll-like receptor (TLR) signals in myeloid dendritic cells (DCs) could differentially stimulate iNKT cells. Together with the lipopolysaccharide-detecting receptor TLR4, activation of the nucleic acid sensors TLR7 and TLR9 in DCs were particularly potent in stimulating iNKT cells to produce IFN-gamma, but not IL-4. iNKT cell activation in response to TLR9 stimulation required combined synthesis of type I interferon and de novo production of charged beta-linked glycosphingolipid(s) by DCs. In addition, DCs stimulated via TLR9 activated both iNKT cells and NK cells in vivo and protected mice against B16F10-induced melanoma metastases. These data underline the role of TLR9 in iNKT cell activation and might have relevance to infectious diseases and cancer.  (+info)

Invariant Valpha14 natural killer T cell activation by edible mushroom acidic glycosphingolipids. (7/12)

Invariant natural killer T (iNKT) cells regulate multi-immune response through Th1/Th2 cytokine release triggered by the recognition of CD1d-restricted glycosphingolipid antigens. Here we report that acidic glycosphingolipids (AGLs) of mushroom (Hypsizigus marmoreus and Pleurotus eryngii) presented by murine CD1d-transfected rat basophilic leukocytes induced interleukin-2 (IL-2) release from iNKT hybridoma cells. AGL-1, one of the AGLs, containing mannose at the non-reducing ends, induced CD1d-dependent IL-2 release. Al-though alpha-galactosylceramide (alpha-GalCer) presented by CD11c-positive cells induced both interferon-gamma (IFN-gamma) and IL-4 release, all of AGLs presented by CD11c-positive cells and AGL-1 presented by B cells induced IL-4 release from iNKT hybridoma cells. A single intravenous injection of AGLs into B6 mice induced only a little elevation of IL-4 in serum but repeated intravenous injection of AGLs induced prolonged retention of IL-4 in serum; therefore, these results suggested that edible mushroom AGLs might contribute to the retention of immunohomeostasis through the minimum induction of iNKT cell activation in vivo.  (+info)

Structure of phosphonoglycosphingolipid containing pyruvylated galactose in nerve fibers of Aplysia kurodai. (8/12)

A phosphonoglycosphingolipid, designated as FGL-IIb, was identified in nerve fibers of Aplysia kurodai by two-dimensional thin layer chromatography (Abe, S., Araki, S., and Satake, M. (1986) Biomed. Res. (Tokyo) 7, 47-51). FGL-IIb was isolated from the nervous system of A. kurodai by Iatrobeads column chromatography using three solvent systems. Pyruvic acid was identified by thin layer chromatography as its 2,4-dinitrophenylhydrazone and established by permethylation studies to be attached as a ketal to O-3 and O-4 of the terminal galactose of the oligosaccharide chain in FGL-IIb. By sugar analysis, permethylation studies, fast atom bombardment-mass spectrometry, and proton magnetic resonance spectrometry, the structure of FGL-IIb was concluded to be [3,4-O-(1-carboxyethylidene)]Gal beta 1----3GalNAc alpha 1----3(Fuc alpha 1----2) (2-aminoethylphosphonyl----6)Gal beta 1----4Glc beta 1----1ceramide. Its major aliphatic components were palmitic acid, octadeca-4-sphingenine and anteisononadeca-4-sphingenine. This is the first report of the occurrence of pyruvylated galactose as a constituent of animal sphingolipid.  (+info)

Acidic glycosphingolipids are a class of complex lipids that contain one or more sugar molecules (glycans) and a fatty acid attached to sphingosine, which is a type of amino alcohol. The term "acidic" refers to the presence of a negatively charged group, such as a sulfate or a carboxylic acid, in the glycan part of the molecule.

Acidic glycosphingolipids are important components of cell membranes and play a role in various biological processes, including cell recognition, signal transduction, and cell adhesion. They are also involved in the development and progression of several diseases, such as cancer, neurodegenerative disorders, and infectious diseases caused by bacteria and viruses.

Examples of acidic glycosphingolipids include sulfatides, gangliosides, and globosides, which differ in the structure and composition of their sugar chains. Abnormalities in the metabolism or function of acidic glycosphingolipids have been associated with various pathological conditions, such as lysosomal storage diseases, inflammatory disorders, and autoimmune diseases.

Glycosphingolipids are a type of complex lipid molecule found in animal cell membranes, particularly in the outer leaflet of the plasma membrane. They consist of a hydrophobic ceramide backbone, which is composed of sphingosine and fatty acids, linked to one or more hydrophilic sugar residues, such as glucose or galactose.

Glycosphingolipids can be further classified into two main groups: neutral glycosphingolipids (which include cerebrosides and gangliosides) and acidic glycosphingolipids (which are primarily gangliosides). Glycosphingolipids play important roles in various cellular processes, including cell recognition, signal transduction, and cell adhesion.

Abnormalities in the metabolism or structure of glycosphingolipids have been implicated in several diseases, such as lysosomal storage disorders (e.g., Gaucher's disease, Fabry's disease) and certain types of cancer (e.g., ganglioside-expressing neuroblastoma).

Sulfoglycosphingolipids are a type of glycosphingolipid that contain a sulfate ester group in their carbohydrate moiety. They are important components of animal cell membranes and play a role in various biological processes, including cell recognition, signal transduction, and cell adhesion.

The most well-known sulfoglycosphingolipids are the sulfatides, which contain a 3'-sulfate ester on the galactose residue of the glycosphingolipid GalCer (galactosylceramide). Sulfatides are abundant in the nervous system and have been implicated in various neurological disorders.

Other sulfoglycosphingolipids include the seminolipids, which contain a 3'-sulfate ester on the galactose residue of lactosylceramide (Galβ1-4Glcβ1-Cer), and are found in high concentrations in the testis.

Abnormalities in sulfoglycosphingolipid metabolism have been associated with several genetic disorders, such as metachromatic leukodystrophy (MLD) and globoid cell leukodystrophy (GLD), which are characterized by progressive neurological deterioration.

Thin-layer chromatography (TLC) is a type of chromatography used to separate, identify, and quantify the components of a mixture. In TLC, the sample is applied as a small spot onto a thin layer of adsorbent material, such as silica gel or alumina, which is coated on a flat, rigid support like a glass plate. The plate is then placed in a developing chamber containing a mobile phase, typically a mixture of solvents.

As the mobile phase moves up the plate by capillary action, it interacts with the stationary phase and the components of the sample. Different components of the mixture travel at different rates due to their varying interactions with the stationary and mobile phases, resulting in distinct spots on the plate. The distance each component travels can be measured and compared to known standards to identify and quantify the components of the mixture.

TLC is a simple, rapid, and cost-effective technique that is widely used in various fields, including forensics, pharmaceuticals, and research laboratories. It allows for the separation and analysis of complex mixtures with high resolution and sensitivity, making it an essential tool in many analytical applications.

A "carbohydrate sequence" refers to the specific arrangement or order of monosaccharides (simple sugars) that make up a carbohydrate molecule, such as a polysaccharide or an oligosaccharide. Carbohydrates are often composed of repeating units of monosaccharides, and the sequence in which these units are arranged can have important implications for the function and properties of the carbohydrate.

For example, in glycoproteins (proteins that contain carbohydrate chains), the specific carbohydrate sequence can affect how the protein is processed and targeted within the cell, as well as its stability and activity. Similarly, in complex carbohydrates like starch or cellulose, the sequence of glucose units can determine whether the molecule is branched or unbranched, which can have implications for its digestibility and other properties.

Therefore, understanding the carbohydrate sequence is an important aspect of studying carbohydrate structure and function in biology and medicine.

Neutral glycosphingolipids (NGSLs) are a type of glycosphingolipid, which are lipids that contain a ceramide backbone with one or more sugar residues attached. NGSLs are characterized by the absence of charged groups in their carbohydrate moiety. They consist of a core structure of ceramide, to which one or more sugars such as glucose or galactose are attached.

NGSLs can be further classified into two main categories: cerebrosides and globosides. Cerebrosides contain a single sugar residue (monosaccharide) attached to the ceramide backbone, while globosides contain more complex oligosaccharide chains. NGSLs are important components of cell membranes and play a role in various biological processes, including cell recognition, signal transduction, and cell adhesion.

Abnormal accumulation of NGSLs can lead to various genetic disorders known as sphingolipidoses, such as Gaucher's disease, Fabry's disease, and Krabbe's disease. These conditions are characterized by the buildup of lipids in various organs and tissues, leading to progressive damage and dysfunction.

Lactosylceramides are a type of glycosphingolipid, which are complex lipids found in the outer layer of cell membranes. They consist of a ceramide molecule (a fatty acid and sphingosine) with a lactose sugar (glucose and galactose) attached. Lactosylceramides play important roles in various cellular processes, including cell recognition, signal transduction, and adhesion. They are also involved in the development and progression of certain diseases, such as cancer and neurological disorders.

Gangliosides are a type of complex lipid molecule known as sialic acid-containing glycosphingolipids. They are predominantly found in the outer leaflet of the cell membrane, particularly in the nervous system. Gangliosides play crucial roles in various biological processes, including cell recognition, signal transduction, and cell adhesion. They are especially abundant in the ganglia (nerve cell clusters) of the peripheral and central nervous systems, hence their name.

Gangliosides consist of a hydrophobic ceramide portion and a hydrophilic oligosaccharide chain that contains one or more sialic acid residues. The composition and structure of these oligosaccharide chains can vary significantly among different gangliosides, leading to the classification of various subtypes, such as GM1, GD1a, GD1b, GT1b, and GQ1b.

Abnormalities in ganglioside metabolism or expression have been implicated in several neurological disorders, including Parkinson's disease, Alzheimer's disease, and various lysosomal storage diseases like Tay-Sachs and Gaucher's diseases. Additionally, certain bacterial toxins, such as botulinum neurotoxin and tetanus toxin, target gangliosides to gain entry into neuronal cells, causing their toxic effects.

Globosides are a type of glycosphingolipids, which are molecules that consist of a lipid and a carbohydrate. They are found in animal tissues, especially in the nervous system. The term "globoside" refers to a specific structure of these molecules, where the carbohydrate portion consists of a complex chain of sugars, including galactose, N-acetylgalactosamine, and glucose. Globosides play important roles in cell recognition and interaction, and abnormalities in their metabolism have been associated with certain diseases, such as paroxysmal nocturnal hemoglobinuria (PNH).

Glucosylceramides are a type of glycosphingolipid, which are complex lipids found in the outer layer of cell membranes. They consist of a ceramide molecule (a fatty acid and sphingosine) with a glucose molecule attached to it through a glycosidic bond.

Glucosylceramides play important roles in various cellular processes, including cell signaling, membrane structure, and cell-to-cell recognition. They are particularly abundant in the nervous system, where they contribute to the formation of the myelin sheath that surrounds nerve fibers.

Abnormal accumulation of glucosylceramides is associated with certain genetic disorders, such as Gaucher disease and Krabbe disease, which are characterized by neurological symptoms and other health problems. Enzyme replacement therapy or stem cell transplantation may be used to treat these conditions.

Cerebrosides are a type of sphingolipid, which are lipids that contain sphingosine. They are major components of the outer layer of cell membranes and are particularly abundant in the nervous system. Cerebrosides are composed of a ceramide molecule (a fatty acid attached to sphingosine) and a sugar molecule, usually either glucose or galactose.

Glycosphingolipids that contain a ceramide with a single sugar residue are called cerebrosides. Those that contain more complex oligosaccharide chains are called gangliosides. Cerebrosides play important roles in cell recognition, signal transduction, and cell adhesion.

Abnormalities in the metabolism of cerebrosides can lead to various genetic disorders, such as Gaucher's disease, Krabbe disease, and Fabry disease. These conditions are characterized by the accumulation of cerebrosides or their breakdown products in various tissues, leading to progressive damage and dysfunction.

Glycolipids are a type of lipid (fat) molecule that contain one or more sugar molecules attached to them. They are important components of cell membranes, where they play a role in cell recognition and signaling. Glycolipids are also found on the surface of some viruses and bacteria, where they can be recognized by the immune system as foreign invaders.

There are several different types of glycolipids, including cerebrosides, gangliosides, and globosides. These molecules differ in the number and type of sugar molecules they contain, as well as the structure of their lipid tails. Glycolipids are synthesized in the endoplasmic reticulum and Golgi apparatus of cells, and they are transported to the cell membrane through vesicles.

Abnormalities in glycolipid metabolism or structure have been implicated in a number of diseases, including certain types of cancer, neurological disorders, and autoimmune diseases. For example, mutations in genes involved in the synthesis of glycolipids can lead to conditions such as Tay-Sachs disease and Gaucher's disease, which are characterized by the accumulation of abnormal glycolipids in cells.

Ceramides are a type of lipid molecule that are found naturally in the outer layer of the skin (the stratum corneum). They play a crucial role in maintaining the barrier function and hydration of the skin. Ceramides help to seal in moisture, support the structure of the skin, and protect against environmental stressors such as pollution and bacteria.

In addition to their role in the skin, ceramides have also been studied for their potential therapeutic benefits in various medical conditions. For example, abnormal levels of ceramides have been implicated in several diseases, including diabetes, cardiovascular disease, and cancer. As a result, ceramide-based therapies are being investigated as potential treatments for these conditions.

Medically, ceramides may be mentioned in the context of skin disorders or diseases where there is a disruption in the skin's barrier function, such as eczema, psoriasis, and ichthyosis. In these cases, ceramide-based therapies may be used to help restore the skin's natural barrier and improve its overall health and appearance.

Sphingolipids are a class of lipids that contain a sphingosine base, which is a long-chain amino alcohol with an unsaturated bond and an amino group. They are important components of animal cell membranes, particularly in the nervous system. Sphingolipids include ceramides, sphingomyelins, and glycosphingolipids.

Ceramides consist of a sphingosine base linked to a fatty acid through an amide bond. They play important roles in cell signaling, membrane structure, and apoptosis (programmed cell death).

Sphingomyelins are formed when ceramides combine with phosphorylcholine, resulting in the formation of a polar head group. Sphingomyelins are major components of the myelin sheath that surrounds nerve cells and are involved in signal transduction and membrane structure.

Glycosphingolipids contain one or more sugar residues attached to the ceramide backbone, forming complex structures that play important roles in cell recognition, adhesion, and signaling. Abnormalities in sphingolipid metabolism have been linked to various diseases, including neurological disorders, cancer, and cardiovascular disease.

"A novel glycosphingolipid-degrading enzyme cleaves the linkage between the oligosaccharide and ceramide of neutral and acidic ... glycosphingolipids". J. Biol. Chem. 261 (30): 14278-82. PMID 3771534. Portal: Biology v t e (EC 3.2.1, Enzymes of known ...
"Mushroom acidic glycosphingolipid induction of cytokine secretion from murine T cells and proliferation of NK1.1 α/β TCR-double ...
In the case of glycosphingolipids, exohydrolases acting at acidic pH optima cause the stepwise release of monosaccharide units ... Constitutive degradation of sphingolipids and glycosphingolipids takes place in the acidic subcellular compartments, the late ... such as sphingomyelin and the complex glycosphingolipids. ...
... s are a sub-class of the lipid class glycosphingolipid with three to nine sugar molecules as the side chain (or R ... a sugar with an acidic carboxy-group. However, some globosides with the core structure Cer-Glc-Gal-Gal do contain neuraminic ... When the globo-series glycosphingolipid (GSL) was reduced in the experiment the ERK signaling from the receptor tyrosine kinase ... acid, e.g. the globo-series glycosphingolipid "SSEA-4-antigen". The side chain can be cleaved by galactosidases and ...
During the digestive process of the bonnethead shark, the acidic stomach weakens the cell walls of the seagrass and allows for ... as it plays an important role in the degradation of glycosphingolipids. The enzyme will break down glucosylceramide into ... while the pH of a human's stomach is acidic (between 1.5 and 3.5). However, humans do require β-glucosidase, Lysosomal β- ...
... glycosphingolipids MeSH D09.400.410.420.025 - acidic glycosphingolipids MeSH D09.400.410.420.025.060 - antigens, cd15 MeSH ... neutral glycosphingolipids MeSH D09.400.410.420.525.200 - ceramides MeSH D09.400.410.420.525.200.250 - cerebrosides MeSH ...
... glycosphingolipids MeSH D10.570.877.360.025 - acidic glycosphingolipids MeSH D10.570.877.360.025.060 - antigens, cd15 MeSH ... acidic glycosphingolipids MeSH D10.390.470.025.060 - antigens, cd15 MeSH D10.390.470.025.475 - gangliosides MeSH D10.390. ... neutral glycosphingolipids MeSH D10.390.470.675.200 - ceramides MeSH D10.390.470.675.200.250 - cerebrosides MeSH D10.390. ... neutral glycosphingolipids MeSH D10.570.877.360.612.200 - ceramides MeSH D10.570.877.360.612.200.250 - cerebrosides MeSH ...
In the presence of psychosine, however, the levels of cAMP increased at a shifted, more acidic pH range. As such, psychosine ... as well as several other related glycosphingolipids. However, the specific binding of psychosine to GPR65 has been contested as ... It was found that overexpression of GPR65 prevents tumor cell death in acidic conditions in vitro and facilitates tumor growth ... After myocardial infarction, anaerobic respiration and severe inflammation occurs-both of which are accompanied by an acidic ...
The Lactosylceramides, also known as LacCer, are a class of glycosphingolipids composed of a variable hydrophobic ceramide ... Gaucher's disease is a sphingolipidosis described by a particular inadequacy in acidic glucocerebrosidase, which results in ... William W., Christie (10 January 2019). "Lactosylceramide and non-Acidic Oligoglycosylceramides". Retrieved 13 July 2019. {{ ...
An acidic pH (around 5.0) and low amounts of water make the epidermis hostile to many microorganic pathogens. Non-pathogenic ... For example glycosphingolipids become ceramides and phospholipids become free fatty acids. spinous layer (stratum spinosum) ... glycosphingolipids, free sterols, phospholipids and catabolic enzymes. Langerhans cells, immunologically active cells, are ...
Srinivas VR, Acharya S, Rawat S, Sharma V; Surolia A (2000). "The primary structure of the acidic lectin from winged bean ( ... Surolia has elucidated the role of glycosphingolipids as biological receptors through studies on lectin-glycolipid interactions ... Patanjali S.R., Sajjan S. U.; Surolia A. (1988). "Erythrocyte-binding studies on an acidic lectin from winged bean ( ...
However, this is only under acidic conditions not neutral conditions. To fully understand the role of sulfatide in the cycle of ... MAL forms complexes with sulfatide and other glycosphingolipids, and these complexes have been shown to play a role in apical ...
This could have immense effects on the signaling functions of ceramide because it is known that ceramide generated by acidic ... 2007). "Glycosphingolipid synthesis requires FAPP2 transfer of glucosylceramide". Nature. 449 (7158): 62-67. Bibcode:2007Natur. ... In addition to their role as building blocks of biological membranes, glycosphingolipids have long attracted attention because ... Glucosylceramides (GluCer) are the most widely distributed glycosphingolipids in cells serving as precursors for the formation ...
"A novel glycosphingolipid-degrading enzyme cleaves the linkage between the oligosaccharide and ceramide of neutral and acidic ... glycosphingolipids". J. Biol. Chem. 261 (30): 14278-82. PMID 3771534. Portal: Biology v t e (EC 3.2.1, Enzymes of known ...
Gb3 glycosphingolipids are the specific receptors for bacterial Shiga toxin. Whereas the trisaccharidic head group of Gb3 ... Indeed, the assembly showed that already the presence of the Lewis acidic TMSOTf proved to be sufficient to cleave PMB after ... Head group labelled glycosphingolipids eventually enabled us to address the question how the fatty acid of a glycosphingolipid ... Glycosphingolipids. Glycosphingolipids (GSLs) are a subclass of glycolipids that are found in cell membranes of various ...
Gangliosides are acidic glycosphingolipids that contribute a substantial presence to the outer leaflet of the cell plasma ...
To characterize biomarkers in neural tumors, we analyzed the acidic lipid fractions of 13 neural tumor cell lines using enzyme- ... Glycosphingolipid Antigens in Neural Tumor Cell Lines and Anti-Glycosphingolipid Antibodies in Sera of Patients with Neural ... Glycosphingolipid Antigens in Neural Tumor Cell Lines and Anti-Glycosphingolipid Antibodies in Sera of Patients with Neural ... These glycosphingolipids (GSLs) were expressed in all cell lines with concentrations ranging from 210 to 330 ng per 2 × 106 ...
Gangliosides are acidic glycosphingolipids that form lipid rafts in the outer leaflet of the cell plasma membrane, especially ... Gangliosides1 are acidic glycosphingolipids that form lipid rafts in the outer leaflet of the cell plasma membrane, especially ... binds several glycosphingolipids including GT1b. GT1b is a scavenger for •OH radicals and protects against brain mtDNA damage, ...
Gangliosides are acidic glycosphingolipids that form lipid rafts in the outer leaflet of the cell plasma membrane, especially ... Gangliosides1 are acidic glycosphingolipids that form lipid rafts in the outer leaflet of the cell plasma membrane, especially ... binds several glycosphingolipids including GT1b. GT1b is a scavenger for •OH radicals and protects against brain mtDNA damage, ...
ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS ACÍDICOS. ACQUIRED HYPEROSTOSIS SYNDROME. ...
ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS NEUTROS. NEUTRAL GLYCOSPHINGOLIPIDS. ...
ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPÍDIOS ACÍDICOS. GLICOESFINGOLIPIDOS NEUTRALES. NEUTRAL GLYCOSPHINGOLIPIDS. ...
ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS ACÍDICOS. ACQUIRED HYPEROSTOSIS SYNDROME. ...
ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS ACÍDICOS. ACQUIRED HYPEROSTOSIS SYNDROME. ...
ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS NEUTROS. NEUTRAL GLYCOSPHINGOLIPIDS. ...
ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS ACÍDICOS. ACQUIRED HYPEROSTOSIS SYNDROME. ...
ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPÍDIOS ACÍDICOS. GLICOESFINGOLIPIDOS NEUTRALES. NEUTRAL GLYCOSPHINGOLIPIDS. ...
ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS NEUTROS. NEUTRAL GLYCOSPHINGOLIPIDS. ...
ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPÍDIOS ACÍDICOS. GLICOESFINGOLIPIDOS NEUTRALES. NEUTRAL GLYCOSPHINGOLIPIDS. ...
ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS ACÍDICOS. ACQUIRED HYPEROSTOSIS SYNDROME. ...
ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS ACÍDICOS. ACQUIRED HYPEROSTOSIS SYNDROME. ...
Acidic glycosphingolipids (gangliosides). Gangliosides contain in their oligosaccharide head groups one or more residues of a ... b) glycosphingolipids.. Lipids usually contain fatty acids as components. Such lipids are called saponifiable lipids since they ... Although glycosphingolipids are only minor constit-uents of membranes, they appear to be extremely important in a number of ... The neutral glycosphingolipids are important cell-surface components in animal tissues. Their nonpolar tails presumably ...
A subclass of ACIDIC GLYCOSPHINGOLIPIDS. They contain one or more sialic acid (N-ACETYLNEURAMINIC ACID) residues. Using the ... and ACIDIC GLYCOSPHINGOLIPIDS which comprises sialosylglycosylsphingolipids (GANGLIOSIDES); SULFOGLYCOSPHINGOLIPIDS (formerly ... Glycosphingolipids. Lipids containing at least one monosaccharide residue and either a sphingoid or a ceramide (CERAMIDES). ... While several glycosphingolipids and some other carbohydrate-containing substances that were tested lacked the ability to mimic ...
3-O-Sulfogalactosylceramide is an acidic, sulfated glycosphingolipid, often known as sulfatide. This lipid occurs in membranes ... Cerebrosides are glycosphingolipids. There are four types of glycosphingolipids, the cerebrosides, sulfatides, globosides and ... Sulfatides are glycosphingolipids. There are four types of glycosphingolipids, the cerebrosides, sulfatides, globosides and ... An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A ...
Basic glycosphingolipids. *Sphingoid bases. *Amphoteric glycosphingolipids. *Neutral glycosphingolipids. *Acidic ...
Acidic glycosphingolipids of the ascidian, Halocynthia roretzi │ 立命館大学理工学研究所紀要 │ 65,101-114 (共著) ... Isolation and characterization of a novel uronic acid-containing acidic glycosphingolipid from the ascidian Halocynthia roretzi ... Characterization of a Novel Rhamnose-containing Acidic Glycosphingolipid from the Ascidian Halocynthia aurantium. │ Journal of ... Novel neogala-series glycosphingolipids with a terminal glucose residue from the fungus Mariannaea elegans │ Biosci. Biotechnol ...
Acidic Residue Modifications. Amino acids with a negatively charged R group at pH 7.0 include aspartic acid and glutamic acid, ... or glycosphingolipids (Hancock and Sahl, 2006). Increased hemolytic activity in most AMP analogs is commonly observed, and it ... Shi, W., He, P., Zeng, X.-C., Wu, W., and Chen, X. (2018). Inhibitory Effect of an Acidic Peptide on the Activity of an ... As aforementioned, AMPs have both acidic and basic amino acids; therefore, they present an amphipathic character that gives ...
... neutral glycosphingolipid Gal-Cer(d18:1/16:0)) and stingray (acidic glycosphingolipid sulpho-Gal-Cer(d18:1/16:0)). Free fatty ... rainbow trout and burbot contain high amounts of neutral and acidic glycosphingolipids as well as sulfoglycolipids. These ... Unknown MS signals could be assigned to different glycosphingolipids in sterlet ( ... particularly sphingomyelin and acidic phospholipids) and the saturation degree of the fatty acyl residues, there were ...
... sphingosine storage leads to a decrease in Ca2+ levels in the acidic compartment and, consequently, fusion/trafficking ... an iminosugar that inhibits glycosphingolipid synthesis) has been approved in the EU for the treatment of progressive ...
In the first, Tamboli and colleagues fed neuroblastoma cells an excess of glycosphingolipids; in the second, the authors used ... the proton pump in lysosomes that maintains their acidic internal environment. Research on LSDs has provided some evidence in ... and the accumulation of APP-CTFs and Aβ in brains of mice with three different glycosphingolipid (GSL) storage diseases ( ... Germany and Belgium report that several inhibitors of vacuolar ATPase boost progranulin levels by increasing the pH of acidic ...
Acidic Glycosphingolipids *Ceramides *Other Glycolipids *Glycosylation Related Products *Inhibitors and Substrates *Glycan ...
Acidic Glycosphingolipids *Ceramides *Other Glycolipids *Glycosylation Related Products *Inhibitors and Substrates *Glycan ...

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