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.
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)
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)
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)
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.
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)
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.
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.
GLYCOSPHINGOLIPIDS with a sulfate group esterified to one of the sugar groups.
The sequence of carbohydrates within POLYSACCHARIDES; GLYCOPROTEINS; and GLYCOLIPIDS.
Glycosphingolipids which contain as their polar head group a trisaccharide (galactose-galactose-glucose) moiety bound in glycosidic linkage to the hydroxyl group of ceramide. Their accumulation in tissue, due to a defect in ceramide trihexosidase, is the cause of angiokeratoma corporis diffusum (FABRY DISEASE).
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 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.
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.
An X-linked inherited metabolic disease caused by a deficiency of lysosomal ALPHA-GALACTOSIDASE A. It is characterized by intralysosomal accumulation of globotriaosylceramide and other GLYCOSPHINGOLIPIDS in blood vessels throughout the body leading to multi-system complications including renal, cardiac, cerebrovascular, and skin disorders.
A mass spectrometric technique that is used for the analysis of a wide range of biomolecules, such as glycoalkaloids, glycoproteins, polysaccharides, and peptides. Positive and negative fast atom bombardment spectra are recorded on a mass spectrometer fitted with an atom gun with xenon as the customary beam. The mass spectra obtained contain molecular weight recognition as well as sequence information.
An analytical method used in determining the identity of a chemical based on its mass using mass analyzers/mass spectrometers.
Fractionation of a vaporized sample as a consequence of partition between a mobile gaseous phase and a stationary phase held in a column. Two types are gas-solid chromatography, where the fixed phase is a solid, and gas-liquid, in which the stationary phase is a nonvolatile liquid supported on an inert solid matrix.
The largest class of organic compounds, including STARCH; GLYCOGEN; CELLULOSE; POLYSACCHARIDES; and simple MONOSACCHARIDES. Carbohydrates are composed of carbon, hydrogen, and oxygen in a ratio of Cn(H2O)n.
An enzyme that catalyzes the hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-galactosides including galactose oligosaccharides, galactomannans, and galactolipids.
The characteristic 3-dimensional shape of a carbohydrate.
Liquid chromatographic techniques which feature high inlet pressures, high sensitivity, and high speed.
Changes in the amounts of various chemicals (neurotransmitters, receptors, enzymes, and other metabolites) specific to the area of the central nervous system contained within the head. These are monitored over time, during sensory stimulation, or under different disease states.
An amino alcohol with a long unsaturated hydrocarbon chain. Sphingosine and its derivative sphinganine are the major bases of the sphingolipids in mammals. (Dorland, 28th ed)
Organic, monobasic acids derived from hydrocarbons by the equivalent of oxidation of a methyl group to an alcohol, aldehyde, and then acid. Fatty acids are saturated and unsaturated (FATTY ACIDS, UNSATURATED). (Grant & Hackh's Chemical Dictionary, 5th ed)
A basic science concerned with the composition, structure, and properties of matter; and the reactions that occur between substances and the associated energy exchange.
The composition, conformation, and properties of atoms and molecules, and their reaction and interaction processes.
A microanalytical technique combining mass spectrometry and gas chromatography for the qualitative as well as quantitative determinations of compounds.
Spectroscopic method of measuring the magnetic moment of elementary particles such as atomic nuclei, protons or electrons. It is employed in clinical applications such as NMR Tomography (MAGNETIC RESONANCE IMAGING).
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.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
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)
A specific monosialoganglioside that accumulates abnormally within the nervous system due to a deficiency of GM1-b-galactosidase, resulting in GM1 gangliosidosis.
A glycolipid, cross-species antigen that induces production of antisheep hemolysin. It is present on the tissue cells of many species but absent in humans. It is found in many infectious agents.
An intermediate in the biosynthesis of cerebrosides. It is formed by reaction of sphingosine with UDP-galactose and then itself reacts with fatty acid-Coenzyme A to form the cerebroside.
Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.
A glycosphingolipid that accumulates due to a deficiency of hexosaminidase A or B (BETA-N-ACETYLHEXOSAMINIDASES), or GM2 activator protein, resulting in GANGLIOSIDOSES, heredity metabolic disorders that include TAY-SACHS DISEASE and SANDHOFF DISEASE.
The major human blood type system which depends on the presence or absence of two antigens A and B. Type O occurs when neither A nor B is present and AB when both are present. A and B are genetic factors that determine the presence of enzymes for the synthesis of certain glycoproteins mainly in the red cell membrane.
Enzymes that catalyze the transfer of glucose from a nucleoside diphosphate glucose to an acceptor molecule which is frequently another carbohydrate. EC 2.4.1.-.
A family of glycoprotein cofactors that are required for the efficient catabolization of SPHINGOLIPIDS by specific acid hydrolases such as GLUCOSYLCERAMIDASE; GALACTOCEREBROSIDASE; BETA-N-ACETYLHEXOSAMINIDASE; and CEREBROSIDE-SULFATASE.
Antigens stimulating the formation of, or combining with heterophile antibodies. They are cross-reacting antigens found in phylogenetically unrelated species.

Glycosphingolipid depletion in fabry disease lymphoblasts with potent inhibitors of glucosylceramide synthase. (1/20)

BACKGROUND: Fabry disease is an inherited X-linked disorder resulting in the loss of activity of the lysosomal hydrolase alpha-galactosidase A and causing the clinical manifestations of renal failure, cerebral vascular disease, and myocardial infarction. The phenotypic expression of this disorder is manifest by the accumulation of glycosphingolipids containing alpha-galactosyl linkages, most prominently globotriaosylceramide. METHODS: Based on quantitative structure activity studies, we recently reported two newly designed glucosylceramide synthase inhibitors based on 1-phenyl-2-palmitoylamino-3-pyrrolidino-1-propanol (P4). These inhibitors, 4'-hydroxy-P4 and ethylenedioxy-P4, were evaluated for their ability to deplete globotriaosylceramide and other glucosylceramide-based lipids in Fabry lymphocytes and were compared with N-butyldeoxynojirimycin, another reported glucosylceramide synthase inhibitor. RESULTS: Concentrations as low as 10 nmol/L of 4'-hydroxy-P4 and ethylenedioxy-P4 resulted in 70 and 80% depletion, respectively, of globotriaosylceramide, with maximal depletion occurring at three days of treatment. There was no impairment of cell growth. In contrast, N-butyldeoxynojirimycin only minimally lowered globotriaosylceramide levels, even at concentrations as high as 10 micromol/L. Globotriaosylceramide depletion was confirmed by the loss of binding of FITC-conjugated verotoxin B subunit to the lymphoblasts. CONCLUSIONS: These findings suggest that selective glucosylceramide synthase inhibitors are highly effective in the depletion of globotriaosylceramide from Fabry cell lines. We suggest that these compounds have potential therapeutic utility in the treatment of Fabry disease.  (+info)

Modifications of glycosphingolipid profile and synthesis in normal rat fibroblasts and in syngeneic neoplastic cells at different subculture stages. (2/20)

Glycosphingolipids are plasma membrane macromolecules involved in diversified recognition functions on the cell surface resulting in modulation of cell adhesion and differentiation. As the in vitro cellular system of the neoplastic cell line SGS/4A and syngeneic normal fibroblasts (FG) represents a useful tool for studies on molecular mechanisms regulating cell adhesion, neoplastic transformation and cellular ageing, we studied the changes of glycosphingolipid and of the enzymes involved in their metabolism in both cultured cells at different subculture stages. The FG subculture progression induces a drastic decrease of total glycosphingolipid content with consistent alterations in the molecular composition. In particular, a significant decrease of GM(3), a slight increase of GD(1a), the disappearance of 'b'-series gangliosides and the drastic reduction of triosylceramides were observed. On the contrary, the increasing number of SGS/4A subcultures, characterized by a specific and different glycosphingolipid composition as compared with FG cells, does not cause modifications. Although glycosyltransferase activity levels quite well parallel the glycosphingolipid patterns and can account for the noted variations, the mRNA expression analysis of two glycosyltransferases suggests that the in vitro cell ageing of normal rat fibroblasts causes drastic changes in the glycosphingolipid profile through the regulation, at either the transcriptional or post-translational level, of some biosynthetic enzymes.  (+info)

Occurrence of ceramides and neutral glycolipids with unusual long-chain base composition in purified rat liver mitochondria. (3/20)

The free ceramide content of rat liver mitochondria was found to be 1.7 nmol/mg protein and outer membranes contained a three-fold higher concentration than inner membranes. The mitochondrial content in neutral glycolipids was 0.6 nmol/mg protein. The long-chain bases found in free ceramides were d18:1 sphingosine, d18:0 3-ketosphinganine and t21:1 phytosphingosine in increasing order. In contrast, 3-ketosphinganine was the only base of glucosylceramide and lactosylceramide of inner membranes, whereas d18:1 sphingosine was the major long-chain base of glucosylceramide of outer membranes.  (+info)

Neutral glycosphingolipid-dependent inactivation of coagulation factor Va by activated protein C and protein S. (4/20)

To test whether neutral glycosphingolipids can serve as anticoagulant cofactors, the effects of incorporation of neutral glycosphingolipids into phospholipid vesicles on anticoagulant and procoagulant reactions were studied. Glucosylceramide (GlcCer), lactosylceramide (LacCer), and globotriaosylceramide (Gb(3)Cer) in vesicles containing phosphatidylserine (PS) and phosphatidylcholine (PC) dose dependently enhanced factor Va inactivation by the anticoagulant factors, activated protein C (APC) and protein S. Addition of GlcCer to PC/PS vesicles enhanced protein S-dependent APC cleavage in factor Va at Arg-506 by 13-fold, whereas PC/PS vesicles alone minimally affected protein S enhancement of this reaction. Incorporation into PC/PS vesicles of GlcCer, LacCer, or Gb(3)Cer, but not galactosylceramide or globotetraosylceramide, dose dependently prolonged factor Xa-1-stage clotting times of normal plasma in the presence of added APC without affecting baseline clotting times in the absence of APC, showing that certain neutral glycosphingolipids enhance anticoagulant but not procoagulant reactions in plasma. Thus, certain neutral glycosphingolipids (e.g. GlcCer, LacCer, and Gb(3)Cer) can enhance anticoagulant activity of APC/protein S by mechanisms that are distinctly different from those of phospholipids alone. We speculate that under some circumstances certain neutral glycosphingolipids either in lipoprotein particles or in cell membranes may help form antithrombotic microdomains that might enhance down-regulation of thrombin by APC in vivo.  (+info)

Aminopropyl solid phase extraction and 2 D TLC of neutral glycosphingolipids and neutral lysoglycosphingolipids. (5/20)

Methods for isolation of neutral lysoglycosphingolipids (n-lyso-GSLs) such as glucosylsphingosine and galactosylsphingosine normally involve mild alkaline or acid hydrolysis followed by multiple chromatography steps, yielding relatively low recoveries of n-lyso-GSLs and neutral glycosphingolipids (n-GSLs). We now describe a new technique for isolating these compounds using one chromatography step, resulting in quantitative recovery of n-GSLs and n-lyso-GSLs. Lipids are extracted using a modified Folch procedure in which recovery is optimized by reextracting the Folch upper phase with water-saturated butanol. The extract is applied to an aminopropyl solid phase column from which both n-GSLs and n-lyso-GSLs elute in the same fraction. Separation is achieved using a new two-dimensional thin-layer chromatography procedure. The usefulness of this technique for biological samples was tested by examining Glc[4,5-(3)H]ceramide and Glc[4,5-(3)H]sphingosine accumulation in metabolically-labeled neurons treated with an inhibitor of lysosomal glucocerebrosidase. Accurate quantification of both lipids was obtained with Glc[4,5-(3)H]ceramide and Glc[4,5-(3)H]sphingosine accumulating at levels of 20 nmol/mg DNA and 40 pmol/mg DNA, respectively. This simple and rapid technique can therefore be used for the analysis of lyso-GSLs and GSLs in the same tissue, which may permit the determination of their metabolic pathways in normal and in pathological tissues, such as those taken from Gaucher and Krabbe's disease patients.  (+info)

Simultaneous quantification of lyso-neutral glycosphingolipids and neutral glycosphingolipids by N-acetylation with [3H]acetic anhydride. (6/20)

We describe a new method that permits quantification in the pmol to nmol range of three lyso-neutral glycosphingolipids (lyso-n-GSLs), glucosylsphingosine (GlcSph), galactosylsphingosine (GalSph), and lactosylsphingosine, in the same sample as neutral glycosphingolipids (n-GSLs). Lyso-n-GSLs and n-GSLs are initially obtained from a crude lipid extract using Sephadex G25 chromatography, followed by their isolation in one fraction, which is devoid of other contaminating lipids, by aminopropyl solid-phase chromatography. Lyso-n-GSLs and n-GSLs are subsequently separated from one another by weak cation exchange chromatography. N-GSLs are then deacylated by strong alkaline hydrolysis, and the N-deacylated-GSLs and lyso-n-GSLs are subsequently N-acetylated using [3H]acetic anhydride. An optimal concentration of 5 mM acetic anhydride was established, which gave >95% N-acetylation. We demonstrate the usefulness of this technique by showing an approximately 40-fold increase of both GlcSph and glucosylceramide in brain tissue from a glucocerebrosidase-deficient mouse, as well as significant lactosylceramide accumulation. The application and optimization of this technique for lyso-n-GSLs and lyso-GSLs will permit their quantification in small amounts of biological tissues, particularly in the GSL storage diseases, such as Gaucher and Krabbe's disease, in which GlcSph and GalSph, respectively, accumulate.  (+info)

Role of multiple drug resistance protein 1 in neutral but not acidic glycosphingolipid biosynthesis. (7/20)

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)

Detection of new anti-neutral glycosphingolipids antibodies and their effects on Trk neurotrophin receptors. (8/20)

We screened sera from patients with various neurological disorders for the presence of anti-neutral glycosphingolipids antibodies and only found them in sera from relapsing polychondritis with limbic encephalitis patients. Neutral glycosphingolipids are resident in membrane lipid rafts where high affinity nerve growth factor (NGF) receptor, Trk is co-localized. Therefore, we examined whether these antibodies influence the action of NGF in NGF-responsive cells. The results strongly suggest that these antibodies enhance NGF-induced Trk autophosphorylation and neurite outgrowth as well as neurofilament M expression. These data strongly indicate that these anti-neutral glycosphingolipids antibodies have a functional impact on NGF-Trk-mediated intracellular signal transduction pathway.  (+info)

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.

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).

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.

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.

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).

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.

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.

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.

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.

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.

Trihexosylceramides are a type of glycosphingolipids, which are complex lipids found in animal tissues. They consist of a ceramide molecule (a sphingosine and fatty acid) with three hexose sugars attached to it in a specific sequence, typically glucose-galactose-galactose.

Trihexosylceramides are further classified into two types based on the type of ceramide they contain: lactosylceramide (Gal-Glc-Cer) and isoglobotrihexosylceramide (GalNAcβ1-4Galβ1-4Glc-Cer).

These lipids are important components of the cell membrane and play a role in various biological processes, including cell recognition, signal transduction, and cell adhesion. Abnormal accumulation of trihexosylceramides has been implicated in certain diseases, such as Gaucher disease and Tay-Sachs disease, which are caused by deficiencies in enzymes involved in their breakdown.

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.

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.

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.

Fabry disease is a rare X-linked inherited lysosomal storage disorder caused by mutations in the GLA gene, which encodes the enzyme alpha-galactosidase A. This enzyme deficiency leads to the accumulation of glycosphingolipids, particularly globotriaosylceramide (Gb3 or GL-3), in various tissues and organs throughout the body. The accumulation of these lipids results in progressive damage to multiple organ systems, including the heart, kidneys, nerves, and skin.

The symptoms of Fabry disease can vary widely among affected individuals, but common manifestations include:

1. Pain: Acroparesthesias (burning or tingling sensations) in the hands and feet, episodic pain crises, chronic pain, and neuropathy.
2. Skin: Angiokeratomas (small, red, rough bumps on the skin), hypohidrosis (decreased sweating), and anhydrosis (absent sweating).
3. Gastrointestinal: Abdominal pain, diarrhea, constipation, nausea, and vomiting.
4. Cardiovascular: Left ventricular hypertrophy (enlargement of the heart muscle), cardiomyopathy, ischemic heart disease, arrhythmias, and valvular abnormalities.
5. Renal: Proteinuria (protein in the urine), hematuria (blood in the urine), chronic kidney disease, and end-stage renal disease.
6. Nervous system: Hearing loss, tinnitus, vertigo, stroke, and cognitive decline.
7. Ocular: Corneal opacities, cataracts, and retinal vessel abnormalities.
8. Pulmonary: Chronic cough, bronchial hyperresponsiveness, and restrictive lung disease.
9. Reproductive system: Erectile dysfunction in males and menstrual irregularities in females.

Fabry disease affects both males and females, but the severity of symptoms is generally more pronounced in males due to the X-linked inheritance pattern. Early diagnosis and treatment with enzyme replacement therapy (ERT) or chaperone therapy can help manage the progression of the disease and improve quality of life.

Fast Atom Bombardment (FAB) Mass Spectrometry is a technique used for determining the mass of ions in a sample. In FAB-MS, the sample is mixed with a matrix material and then bombarded with a beam of fast atoms, usually xenon or cesium. This bombardment leads to the formation of ions from the sample which can then be detected and measured using a mass analyzer. The resulting mass spectrum provides information about the molecular weight and structure of the sample molecules. FAB-MS is particularly useful for the analysis of large, thermally labile, or polar molecules that may not ionize well by other methods.

Mass spectrometry (MS) is an analytical technique used to identify and quantify the chemical components of a mixture or compound. It works by ionizing the sample, generating charged molecules or fragments, and then measuring their mass-to-charge ratio in a vacuum. The resulting mass spectrum provides information about the molecular weight and structure of the analytes, allowing for identification and characterization.

In simpler terms, mass spectrometry is a method used to determine what chemicals are present in a sample and in what quantities, by converting the chemicals into ions, measuring their masses, and generating a spectrum that shows the relative abundances of each ion type.

Chromatography, gas (GC) is a type of chromatographic technique used to separate, identify, and analyze volatile compounds or vapors. In this method, the sample mixture is vaporized and carried through a column packed with a stationary phase by an inert gas (carrier gas). The components of the mixture get separated based on their partitioning between the mobile and stationary phases due to differences in their adsorption/desorption rates or solubility.

The separated components elute at different times, depending on their interaction with the stationary phase, which can be detected and quantified by various detection systems like flame ionization detector (FID), thermal conductivity detector (TCD), electron capture detector (ECD), or mass spectrometer (MS). Gas chromatography is widely used in fields such as chemistry, biochemistry, environmental science, forensics, and food analysis.

Carbohydrates are a major nutrient class consisting of organic compounds that primarily contain carbon, hydrogen, and oxygen atoms. They are classified as saccharides, which include monosaccharides (simple sugars), disaccharides (double sugars), oligosaccharides (short-chain sugars), and polysaccharides (complex carbohydrates).

Monosaccharides, such as glucose, fructose, and galactose, are the simplest form of carbohydrates. They consist of a single sugar molecule that cannot be broken down further by hydrolysis. Disaccharides, like sucrose (table sugar), lactose (milk sugar), and maltose (malt sugar), are formed from two monosaccharide units joined together.

Oligosaccharides contain a small number of monosaccharide units, typically less than 20, while polysaccharides consist of long chains of hundreds to thousands of monosaccharide units. Polysaccharides can be further classified into starch (found in plants), glycogen (found in animals), and non-starchy polysaccharides like cellulose, chitin, and pectin.

Carbohydrates play a crucial role in providing energy to the body, with glucose being the primary source of energy for most cells. They also serve as structural components in plants (cellulose) and animals (chitin), participate in various metabolic processes, and contribute to the taste, texture, and preservation of foods.

Alpha-galactosidase is an enzyme that breaks down complex carbohydrates, specifically those containing alpha-galactose molecules. This enzyme is found in humans, animals, and microorganisms. In humans, a deficiency of this enzyme can lead to a genetic disorder known as Fabry disease, which is characterized by the accumulation of these complex carbohydrates in various tissues and organs, leading to progressive damage. Alpha-galactosidase is also used as a medication for the treatment of Fabry disease, where it is administered intravenously to help break down the accumulated carbohydrates and alleviate symptoms.

Carbohydrate conformation refers to the three-dimensional shape and structure of a carbohydrate molecule. Carbohydrates, also known as sugars, can exist in various conformational states, which are determined by the rotation of their component bonds and the spatial arrangement of their functional groups.

The conformation of a carbohydrate molecule can have significant implications for its biological activity and recognition by other molecules, such as enzymes or antibodies. Factors that can influence carbohydrate conformation include the presence of intramolecular hydrogen bonds, steric effects, and intermolecular interactions with solvent molecules or other solutes.

In some cases, the conformation of a carbohydrate may be stabilized by the formation of cyclic structures, in which the hydroxyl group at one end of the molecule forms a covalent bond with the carbonyl carbon at the other end, creating a ring structure. The most common cyclic carbohydrates are monosaccharides, such as glucose and fructose, which can exist in various conformational isomers known as anomers.

Understanding the conformation of carbohydrate molecules is important for elucidating their biological functions and developing strategies for targeting them with drugs or other therapeutic agents.

High-performance liquid chromatography (HPLC) is a type of chromatography that separates and analyzes compounds based on their interactions with a stationary phase and a mobile phase under high pressure. The mobile phase, which can be a gas or liquid, carries the sample mixture through a column containing the stationary phase.

In HPLC, the mobile phase is a liquid, and it is pumped through the column at high pressures (up to several hundred atmospheres) to achieve faster separation times and better resolution than other types of liquid chromatography. The stationary phase can be a solid or a liquid supported on a solid, and it interacts differently with each component in the sample mixture, causing them to separate as they travel through the column.

HPLC is widely used in analytical chemistry, pharmaceuticals, biotechnology, and other fields to separate, identify, and quantify compounds present in complex mixtures. It can be used to analyze a wide range of substances, including drugs, hormones, vitamins, pigments, flavors, and pollutants. HPLC is also used in the preparation of pure samples for further study or use.

Brain chemistry refers to the chemical processes that occur within the brain, particularly those involving neurotransmitters, neuromodulators, and neuropeptides. These chemicals are responsible for transmitting signals between neurons (nerve cells) in the brain, allowing for various cognitive, emotional, and physical functions.

Neurotransmitters are chemical messengers that transmit signals across the synapse (the tiny gap between two neurons). Examples of neurotransmitters include dopamine, serotonin, norepinephrine, GABA (gamma-aminobutyric acid), and glutamate. Each neurotransmitter has a specific role in brain function, such as regulating mood, motivation, attention, memory, and movement.

Neuromodulators are chemicals that modify the effects of neurotransmitters on neurons. They can enhance or inhibit the transmission of signals between neurons, thereby modulating brain activity. Examples of neuromodulators include acetylcholine, histamine, and substance P.

Neuropeptides are small protein-like molecules that act as neurotransmitters or neuromodulators. They play a role in various physiological functions, such as pain perception, stress response, and reward processing. Examples of neuropeptides include endorphins, enkephalins, and oxytocin.

Abnormalities in brain chemistry can lead to various neurological and psychiatric conditions, such as depression, anxiety disorders, schizophrenia, Parkinson's disease, and Alzheimer's disease. Understanding brain chemistry is crucial for developing effective treatments for these conditions.

Sphingosine is not a medical term per se, but rather a biological compound with importance in the field of medicine. It is a type of sphingolipid, a class of lipids that are crucial components of cell membranes. Sphingosine itself is a secondary alcohol with an amino group and two long-chain hydrocarbons.

Medically, sphingosine is significant due to its role as a precursor in the synthesis of other sphingolipids, such as ceramides, sphingomyelins, and gangliosides, which are involved in various cellular processes like signal transduction, cell growth, differentiation, and apoptosis (programmed cell death).

Moreover, sphingosine-1-phosphate (S1P), a derivative of sphingosine, is an important bioactive lipid mediator that regulates various physiological functions, including immune response, vascular maturation, and neuronal development. Dysregulation of S1P signaling has been implicated in several diseases, such as cancer, inflammation, and cardiovascular disorders.

In summary, sphingosine is a crucial biological compound with medical relevance due to its role as a precursor for various sphingolipids involved in cellular processes and as a precursor for the bioactive lipid mediator S1P.

Fatty acids are carboxylic acids with a long aliphatic chain, which are important components of lipids and are widely distributed in living organisms. They can be classified based on the length of their carbon chain, saturation level (presence or absence of double bonds), and other structural features.

The two main types of fatty acids are:

1. Saturated fatty acids: These have no double bonds in their carbon chain and are typically solid at room temperature. Examples include palmitic acid (C16:0) and stearic acid (C18:0).
2. Unsaturated fatty acids: These contain one or more double bonds in their carbon chain and can be further classified into monounsaturated (one double bond) and polyunsaturated (two or more double bonds) fatty acids. Examples of unsaturated fatty acids include oleic acid (C18:1, monounsaturated), linoleic acid (C18:2, polyunsaturated), and alpha-linolenic acid (C18:3, polyunsaturated).

Fatty acids play crucial roles in various biological processes, such as energy storage, membrane structure, and cell signaling. Some essential fatty acids cannot be synthesized by the human body and must be obtained through dietary sources.

In the context of medicine, "chemistry" often refers to the field of study concerned with the properties, composition, and structure of elements and compounds, as well as their reactions with one another. It is a fundamental science that underlies much of modern medicine, including pharmacology (the study of drugs), toxicology (the study of poisons), and biochemistry (the study of the chemical processes that occur within living organisms).

In addition to its role as a basic science, chemistry is also used in medical testing and diagnosis. For example, clinical chemistry involves the analysis of bodily fluids such as blood and urine to detect and measure various substances, such as glucose, cholesterol, and electrolytes, that can provide important information about a person's health status.

Overall, chemistry plays a critical role in understanding the mechanisms of diseases, developing new treatments, and improving diagnostic tests and techniques.

Chemical phenomena refer to the changes and interactions that occur at the molecular or atomic level when chemicals are involved. These phenomena can include chemical reactions, in which one or more substances (reactants) are converted into different substances (products), as well as physical properties that change as a result of chemical interactions, such as color, state of matter, and solubility. Chemical phenomena can be studied through various scientific disciplines, including chemistry, biochemistry, and physics.

Gas Chromatography-Mass Spectrometry (GC-MS) is a powerful analytical technique that combines the separating power of gas chromatography with the identification capabilities of mass spectrometry. This method is used to separate, identify, and quantify different components in complex mixtures.

In GC-MS, the mixture is first vaporized and carried through a long, narrow column by an inert gas (carrier gas). The various components in the mixture interact differently with the stationary phase inside the column, leading to their separation based on their partition coefficients between the mobile and stationary phases. As each component elutes from the column, it is then introduced into the mass spectrometer for analysis.

The mass spectrometer ionizes the sample, breaks it down into smaller fragments, and measures the mass-to-charge ratio of these fragments. This information is used to generate a mass spectrum, which serves as a unique "fingerprint" for each compound. By comparing the generated mass spectra with reference libraries or known standards, analysts can identify and quantify the components present in the original mixture.

GC-MS has wide applications in various fields such as forensics, environmental analysis, drug testing, and research laboratories due to its high sensitivity, specificity, and ability to analyze volatile and semi-volatile compounds.

Magnetic Resonance Spectroscopy (MRS) is a non-invasive diagnostic technique that provides information about the biochemical composition of tissues, including their metabolic state. It is often used in conjunction with Magnetic Resonance Imaging (MRI) to analyze various metabolites within body tissues, such as the brain, heart, liver, and muscles.

During MRS, a strong magnetic field, radio waves, and a computer are used to produce detailed images and data about the concentration of specific metabolites in the targeted tissue or organ. This technique can help detect abnormalities related to energy metabolism, neurotransmitter levels, pH balance, and other biochemical processes, which can be useful for diagnosing and monitoring various medical conditions, including cancer, neurological disorders, and metabolic diseases.

There are different types of MRS, such as Proton (^1^H) MRS, Phosphorus-31 (^31^P) MRS, and Carbon-13 (^13^C) MRS, each focusing on specific elements or metabolites within the body. The choice of MRS technique depends on the clinical question being addressed and the type of information needed for diagnosis or monitoring purposes.

Molecular sequence data refers to the specific arrangement of molecules, most commonly nucleotides in DNA or RNA, or amino acids in proteins, that make up a biological macromolecule. This data is generated through laboratory techniques such as sequencing, and provides information about the exact order of the constituent molecules. This data is crucial in various fields of biology, including genetics, evolution, and molecular biology, allowing for comparisons between different organisms, identification of genetic variations, and studies of gene function and regulation.

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.

The Forssman antigen is a type of heterophile antigen, which is a substance that can stimulate an immune response in animals of different species. It was first discovered by the Swedish bacteriologist, John Forssman, in 1911. The Forssman antigen is found in a variety of tissues and organs, including the kidney, liver, and brain, in many different animal species, including humans.

The Forssman antigen is unique because it can induce the production of antibodies that cross-react with tissues from other species. This means that an immune response to the Forssman antigen in one species can also recognize and react with similar antigens in another species, leading to the possibility of cross-species immune reactions.

The Forssman antigen is a complex glycosphingolipid molecule that is found on the surface of cells. It is not clear what role, if any, the Forssman antigen plays in normal physiological processes. However, its presence has been implicated in various disease processes, including autoimmune disorders and transplant rejection.

In summary, the Forssman antigen is a heterophile antigen found in a variety of tissues and organs in many different animal species, including humans. It can induce cross-reacting antibodies and has been implicated in various disease processes.

I'm sorry for any confusion, but "psychosine" is not a commonly used term in medicine or psychology. It is a lipid molecule that has been researched in the context of certain neurological conditions, particularly Krabbe disease, which is a rare and fatal genetic disorder affecting the nervous system.

In Krabbe disease, psychosine accumulates in the body due to a deficiency of an enzyme called galactocerebrosidase. This buildup of psychosine is thought to contribute to the damage and destruction of nerve cells, leading to the symptoms of the disease. However, it's important to note that this is still an area of ongoing research, and there is no medical definition for "psychosine" in a general sense.

"Cattle" is a term used in the agricultural and veterinary fields to refer to domesticated animals of the genus *Bos*, primarily *Bos taurus* (European cattle) and *Bos indicus* (Zebu). These animals are often raised for meat, milk, leather, and labor. They are also known as bovines or cows (for females), bulls (intact males), and steers/bullocks (castrated males). However, in a strict medical definition, "cattle" does not apply to humans or other animals.

The ABO blood-group system is a classification system used in blood transfusion medicine to determine the compatibility of donated blood with a recipient's blood. It is based on the presence or absence of two antigens, A and B, on the surface of red blood cells (RBCs), as well as the corresponding antibodies present in the plasma.

There are four main blood types in the ABO system:

1. Type A: These individuals have A antigens on their RBCs and anti-B antibodies in their plasma.
2. Type B: They have B antigens on their RBCs and anti-A antibodies in their plasma.
3. Type AB: They have both A and B antigens on their RBCs but no natural antibodies against either A or B antigens.
4. Type O: They do not have any A or B antigens on their RBCs, but they have both anti-A and anti-B antibodies in their plasma.

Transfusing blood from a donor with incompatible ABO antigens can lead to an immune response, causing the destruction of donated RBCs and potentially life-threatening complications such as acute hemolytic transfusion reaction. Therefore, it is crucial to match the ABO blood type between donors and recipients before performing a blood transfusion.

Glucosyltransferases (GTs) are a group of enzymes that catalyze the transfer of a glucose molecule from an activated donor to an acceptor molecule, resulting in the formation of a glycosidic bond. These enzymes play crucial roles in various biological processes, including the biosynthesis of complex carbohydrates, cell wall synthesis, and protein glycosylation. In some cases, GTs can also contribute to bacterial pathogenesis by facilitating the attachment of bacteria to host tissues through the formation of glucans, which are polymers of glucose molecules.

GTs can be classified into several families based on their sequence similarities and catalytic mechanisms. The donor substrates for GTs are typically activated sugars such as UDP-glucose, TDP-glucose, or GDP-glucose, which serve as the source of the glucose moiety that is transferred to the acceptor molecule. The acceptor can be a wide range of molecules, including other sugars, proteins, lipids, or small molecules.

In the context of human health and disease, GTs have been implicated in various pathological conditions, such as cancer, inflammation, and microbial infections. For example, some GTs can modify proteins on the surface of cancer cells, leading to increased cell proliferation, migration, and invasion. Additionally, GTs can contribute to bacterial resistance to antibiotics by modifying the structure of bacterial cell walls or by producing biofilms that protect bacteria from host immune responses and antimicrobial agents.

Overall, Glucosyltransferases are essential enzymes involved in various biological processes, and their dysregulation has been associated with several human diseases. Therefore, understanding the structure, function, and regulation of GTs is crucial for developing novel therapeutic strategies to target these enzymes and treat related pathological conditions.

Sphingolipid activator proteins (SAPs), also known as saposins, are a group of small proteins that play a crucial role in the metabolism of sphingolipids, a class of lipids found in cell membranes. These proteins are produced by the cleavage of a precursor protein called prosaposin.

SAPs facilitate the hydrolysis of sphingolipids by activating specific lysosomal hydrolases, enzymes that break down these lipids into simpler molecules. Each SAP has a unique structure and function, and they are named SapA, SapB, SapC, and SapD.

SapA and SapB activate the enzyme glucocerebrosidase, which breaks down glucosylceramide into glucose and ceramide. SapC activates the enzyme galactocerebrosidase, which breaks down galactosylceramide into galactose and ceramide. SapD has multiple functions, including activating the enzyme acid sphingomyelinase, which breaks down sphingomyelin into ceramide and phosphorylcholine.

Deficiencies in SAPs can lead to lysosomal storage disorders, such as Gaucher disease (caused by a deficiency in glucocerebrosidase) and Krabbe disease (caused by a deficiency in galactocerebrosidase). These disorders are characterized by the accumulation of undigested sphingolipids in various tissues, leading to cell dysfunction and tissue damage.

Heterophile antigens are a type of antigen that can induce an immune response in multiple species, not just the one they originate from. They are called "heterophile" because they exhibit cross-reactivity with antibodies produced against different antigens from other species. A common example of heterophile antigens is the Forssman antigen, which can be found in various animals such as guinea pigs, rabbits, and humans.

Heterophile antibody tests are often used in diagnostic medicine to detect certain infections or autoimmune disorders. One well-known example is the Paul-Bunnell test, which was historically used to diagnose infectious mononucleosis (IM) caused by the Epstein-Barr virus (EBV). The test detects heterophile antibodies produced against EBV antigens that cross-react with sheep red blood cells. However, this test has been largely replaced by more specific and sensitive EBV antibody tests.

It is important to note that heterophile antibody tests can sometimes produce false positive results due to the presence of these cross-reactive antibodies in individuals who have not been infected with the targeted pathogen. Therefore, it is crucial to interpret test results cautiously and consider them alongside clinical symptoms, medical history, and other diagnostic findings.

In general, glycosphingolipids can be categorized into two groups: neutral glycosphingolipids (also called glycosphingolipids) ... Neutral glycosphingolipids are also important, for example as blood type antigens. Inheritable enzyme defects can lead to ... Glycosphingolipids are a group of lipids (more specifically, sphingolipids) and are a part of the cell membrane. They consist ... Glycosphingolipids are a subtype of glycolipids containing the amino alcohol sphingosine. They may be considered as ...
"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 ...
It is a recombinant human α-galactosidase-A. It is a hydrolytic lysosomal neutral glycosphingolipid-specific enzyme. The most ...
"Structure of a neutral glycosphingolipid recognized by human antibodies in polyagglutinable erythrocytes from the rare NOR ... "Structure of a neutral glycosphingolipid recognized by human antibodies in polyagglutinable erythrocytes from the rare NOR ... Lisowska, Elwira; Duk, Maria (2004-06-15). "Polyagglutination NOR: new glycosphingolipid antigens recognized by a new type of ...
"Structure of a neutral glycosphingolipid recognized by human antibodies in polyagglutinable erythrocytes from the rare NOR ... The P1PK blood group system consists of three glycosphingolipid antigens: Pk, P1 and NOR. In addition to glycosphingolipids, ... The rare NOR phenotype is caused by the presence of unique NOR1 and NOR2 glycosphingolipid antigens, terminating with Galα1 → ... Shiga Toxin Binds Human Platelets Via Globotriaoslyceramide (Pk antigen) and a Novel Platelet Glycosphingolipid. Infect Immun ...
... is an enzyme that catalyzes a chemical reaction that cleaves gangliosides and neutral glycosphingolipids, releasing fatty acids ... The systematic name of this enzyme class is glycosphingolipid amidohydrolase. This enzyme is also called glycosphingolipid ... Hirabayashi Y, Kimura M, Matsumoto M, Yamamoto K, Kadowaki S, Tochikura T (January 1988). "A novel glycosphingolipid ... In enzymology, a glycosphingolipid deacylase (EC 3.5.1.69) ... the fatty acid and sphingosine base in glycosphingolipids". ...
... s are a sub-class of the lipid class glycosphingolipid with three to nine sugar molecules as the side chain (or R ... Another important characteristic of globosides is that they are neutral at pH 7, because they usually do not contain neuraminic ... When the globo-series glycosphingolipid (GSL) was reduced in the experiment the ERK signaling from the receptor tyrosine kinase ... the globo-series glycosphingolipid "SSEA-4-antigen". The side chain can be cleaved by galactosidases and glucosidases. The ...
... neutral glycosphingolipids MeSH D09.400.410.420.525.200 - ceramides MeSH D09.400.410.420.525.200.250 - cerebrosides MeSH ... glycosphingolipids MeSH D09.400.410.420.025 - acidic glycosphingolipids MeSH D09.400.410.420.025.060 - antigens, cd15 MeSH ...
... 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 ... 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. ...
Sph can also be formed in the extracellular (outer leaflet) side of the plasma membrane by the action of neutral CDase enzyme. ... 2007). "Glycosphingolipid synthesis requires FAPP2 transfer of glucosylceramide". Nature. 449 (7158): 62-67. Bibcode:2007Natur. ... C1P carry ionic charge at neutral pH and contain two hydrophobic chains making it relatively insoluble in aqueous environment. ... In addition to their role as building blocks of biological membranes, glycosphingolipids have long attracted attention because ...
Human neutral ceramidase (nCDase) is an enzyme that plays a critical role in colon cancer and there are currently no potent or ... Ceramidase (EC 3.5.1.23, acylsphingosine deacylase, glycosphingolipid ceramide deacylase) is an enzyme which cleaves fatty ... Presently, 7 human ceramidases encoded by 7 distinct genes have been cloned: acid ceramidase (ASAH1) - cell survival neutral ... "Identification of Small-Molecule Inhibitors of Neutral Ceramidase (nCDase) via Target-Based High-Throughput Screening". SLAS ...
... were found to be elevated in neutral to acidic extracellular pH (pH 7.0-6.5) in cells expressing GPR65. In cells with mutated ... as well as several other related glycosphingolipids. However, the specific binding of psychosine to GPR65 has been contested as ...
Myerowitz R (1997). "Tay-Sachs disease-causing mutations and neutral polymorphisms in the Hex A gene". Human Mutation. 9 (3): ... Lachmann RH, Platt FM (2001). "Substrate reduction therapy for glycosphingolipid storage disorders". Expert Opinion on ... ISBN 978-0-08-049030-4. Kimura, Motoo (1983). The Neutral Theory of Molecular Evolution. Cambridge: Cambridge University Press ... while neutralists favor a form of Motoo Kimura's neutral theory of molecular evolution, which emphasizes the role of genetic ...
It is a neutral, stable naturally occurring compound. N-Acetylmannosamine is also known as N-Acetyl-D-mannosamine monohydrate ... GNE myopathy is a rare genetic disorder caused by hyposialylated muscle proteins and glycosphingolipids because there is ... "Non-specific accumulation of glycosphingolipids in GNE myopathy". Journal of Inherited Metabolic Disease. 37 (2): -297-308. doi ...
EC 3.4.24.15, thimet oligopeptidase EC 3.4.99.32: Transferred entry: Armillaria mellea neutral proteinase. Now EC 3.4.24.20, ... glycosphingolipid deacylase EC 3.5.1.70: aculeacin-A deacylase EC 3.5.1.71: N-feruloylglycine deacylase EC 3.5.1.72: D- ... lens neutral proteinase. Now included with EC 3.4.22.53 (calpain-2) and EC 3.4.25.1 (proteasome endopeptidase complex) EC 3.4. ...
Some of these lipid pathways are extremely complex, for example the mammalian glycosphingolipid pathway. The establishment of ... "Qualitative Analysis and Quantitative Assessment of Changes in Neutral Glycerol Lipid Molecular Species within Cells". ...
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 ...
In general, glycosphingolipids can be categorized into two groups: neutral glycosphingolipids (also called glycosphingolipids) ... Neutral glycosphingolipids are also important, for example as blood type antigens. Inheritable enzyme defects can lead to ... Glycosphingolipids are a group of lipids (more specifically, sphingolipids) and are a part of the cell membrane. They consist ... Glycosphingolipids are a subtype of glycolipids containing the amino alcohol sphingosine. They may be considered as ...
Gb3 glycosphingolipids are the specific receptors for bacterial Shiga toxin. Whereas the trisaccharidic head group of Gb3 ... In contrast, globosides are neutral lipid molecules.. For the analysis of GSLs, they are extracted from tissues and cells using ... 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 ...
Glycosphingolipid Disorders of the Brain. Stephanie D. Boomkamp, Terry D. Butters. 18. Role of Neutral Sphingomyelinases in ...
Minor brain neutral glycosphingolipids; cellular topography and alterations in dys‐and demyelination [E. L. Hogan]. Medical ... Extended globo-series glycosphingolipids that mediate cell adhesion and signaling. [S. Hakomori]. Pacific Northwest Research ... Induction of glycosphingolipid biosynthesis and neurite outgrowth of primary cultured neurons by L-threo-1-phenyl-2- ... Molecular mechanisms of TNF-induced activation of neutral sphingomyelinase [M.Kronke]. University of Kiel, GeImany, ...
This inborn error of metabolism results in unremitting deposition of neural glycosphingolipids in the lysosomes of the vascular ... results in the insidious storage of neutral glycosphingolipids, most notably globotriaosylceramide (G3b). Angiokeratoma ... leads to lysosomal accumulation of neutral glycosphingolipids, most notably globotriaosylceramide (G3b). These ... Deposition of glycosphingolipids can be attributed to both endogenous production and diffusion of material from the circulation ...
Accumulation of globotriaosylceramide (Gb3) and other neutral glycosphingolipids with galactosyl residues is the hallmark of ... Cholesterol facilitates interactions between α-synuclein oligomers and charge-neutral membranes.. Febs Lett. 2015; 589(19): ... Non-PEGylated cationic nanocarriers have previously shown little to no diffusion in the vitreous, whilst neutral and anionic ... Host membrane glycosphingolipids and lipid microdomains facilitate Histoplasma capsulatum internalization by macrophages. ...
... a myelin-enriched glycosphingolipid), neutral phospholipids, or GA1 in the treated mice. Our findings indicate that the effects ...
Some of the neutral glycosphingolipids are found on the surface of red blood cells and give them blood-group specificity. ... The neutral glycosphingolipids are important cell-surface components in animal tissues. Their nonpolar tails presumably ... they are called neutral glycosphingolipids. The simplest of these are the cerebrosides, which contain as their polar head group ... Fatty acid esters of the alcohol glycerol are called acylglycerols or glycerides; they are sometimes re-ferred to as "neutral ...
NEUTRAL GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS NEUTRALES. GLICOESFINGOLIPÍDIOS NEUTROS. NICORANDIL. NICORANDIL. NICORANDIL. ... 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. ...
NEUTRAL GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS NEUTRALES. GLICOESFINGOLIPÍDIOS NEUTROS. NICORANDIL. NICORANDIL. NICORANDIL. ... ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS ACÍDICOS. ACQUIRED HYPEROSTOSIS SYNDROME. ...
NEUTRAL GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS NEUTRALES. GLICOESFINGOLIPÍDIOS NEUTROS. NICORANDIL. NICORANDIL. NICORANDIL. ... ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS ACÍDICOS. ACQUIRED HYPEROSTOSIS SYNDROME. ...
ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS NEUTROS. NEUTRAL GLYCOSPHINGOLIPIDS. ...
NEUTRAL GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS NEUTRALES. GLICOESFINGOLIPÍDIOS NEUTROS. NICORANDIL. NICORANDIL. NICORANDIL. ... 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. ...
NEUTRAL GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS NEUTRALES. GLICOESFINGOLIPÍDIOS NEUTROS. NICORANDIL. NICORANDIL. NICORANDIL. ... ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS ACÍDICOS. ACQUIRED HYPEROSTOSIS SYNDROME. ...
NEUTRAL GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS NEUTRALES. GLICOESFINGOLIPÍDIOS NEUTROS. NICORANDIL. NICORANDIL. NICORANDIL. ... ACIDIC GLYCOSPHINGOLIPIDS. GLICOESFINGOLIPIDOS ACIDICOS. GLICOESFINGOLIPÍDIOS ACÍDICOS. ACQUIRED HYPEROSTOSIS SYNDROME. ...
Neutral Glycosphingolipids [D09.400.410.420.525]. *Ceramides [D09.400.410.420.525.200]. *Cerebrosides [D09.400.410.420.525.200. ...
Neutral Protease : 0.008% DMEM/F12 Hussein, D. , Punjaruk, W. , Storer, L. , Shaw, L. , Othman, R. , Ottoman, R. , Peet, A. , ... Glycosphingolipid Antigens in Cultured Microvasular Bovine Brain Endothelial Cells: Sulfoglucuronosyl Paragloboside as a Target ...
Globosides are the major neutral glycosphingolipid in normal kidneys and erythrocytes.. ...
... neutral glycosphingolipids, mainly globotriaosylceramide (Gb3), accumulate in a variety of cells and tissues, leading to a wide ... intralysosomal deposition of undegraded glycosphingolipid products, primarily Gb3, in multiple organs [1, 2]. The initial signs ...
Members of the class of neutral glycosphingolipids. They are the basic units of SPHINGOLIPIDS. They are sphingoids attached via ... They are subdivided into NEUTRAL GLYCOSPHINGOLIPIDS comprising monoglycosyl- and oligoglycosylsphingoids and monoglycosyl- and ... 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 ...
Structural characterization of neutral glycosphingolipids from 3T3-L1 adipocytes. │ Lipids │ 50,913-917 (共著) ... Structural analysis of neutral glycosphingolipids from the silkworm Bombyx mori and the difference in ceramide composition ... Biochemical Studies on Sphingolipids of Artemia franciscana: Novel Neutral Glycosphingolipids │ J. Lipid Res. │ 52 (3),308-327 ... Biochemical studies on sphingolipids of Artemia franciscana: complex neutral glycosphingolipids │ Glycoconjugate Journal │ ...
Rotaviruses specifically bind to the neutral glycosphingolipid asialo-GM1. 1990. Willoughby et al.. 2168971. ... Glycosphingolipid binding specificities of rotavirus: identification of a sialic acid-binding epitope.. 2001. Delorme et al.. ... Multiple glycosphingolipids determine the tissue tropism of parvovirus B19.. 1995. Cooling et al.. 7594654. ...
Basic glycosphingolipids. *Sphingoid bases. *Amphoteric glycosphingolipids. *Neutral glycosphingolipids. *Acidic ...
There are four classifications of glycosphingolipids:. *Neutral glycosphingolipids: 1 or more uncharged sugars (i.e. glucose, ... Acid glycosphingolipids: ionized functional groups (i.e. phosphate or sulfate) attached to neutral or charged sugars[2] ... Glycosphingolipids. Glycosphingolipids have one or more carbohydrate groups in a glycosidic linkage (usually; ceramide ... Sphingolipids can exist in cationic and neutral forms.[2]. There are three major phases that a lipid membrane can take (Figure ...
Those molecules are called neutral glycosphingolipids or cerebrosides because they are often found in the membranes of the ... neutral polar: may react with the solvent. They are polar because R is very small *cysteine: they bind together via S bonds ... As the proteins are charged in function of the pH of the solvent, it will move until its charge becomes neutral. After that, we ... The structure is stable in neutral conditions but if the pH changes, there can be a lot of nearby COO-, what is unstable. ...
  • The chemical reactions and pathways involving globosides, globotetraosylceramides, ceramides containing a core structure of GalNAc-beta-(1,3)-Gal-alpha(1,4)Glc(I). Globosides are the major neutral glycosphingolipid in normal kidneys and erythrocytes. (yeastrc.org)
  • Glycosphingolipids are a group of lipids (more specifically, sphingolipids) and are a part of the cell membrane. (wikipedia.org)
  • Accumulation of neutral lipids, such as triglycerides, in key insulin target tissues, has been postulated to inhibit metabolic functions, however, they are less likely to be deleterious. (frontiersin.org)
  • Glycosphingolipids include: Cerebrosides Gangliosides Globosides Gangliosides are mainly found in the cell membranes of the central nervous system, where their carbohydrate group is responsible for the interaction between individual cells and for signaling. (wikipedia.org)
  • Neutral glycosphingolipids are also important, for example as blood type antigens. (wikipedia.org)
  • The NOR phenotype-related antigens are unique neutral glycosphingolipids recognized by these antibodies and Griffonia simplicifolia IB4 isolectin (GSL-IB4). (slu.se)
  • In contrast, globosides are neutral lipid molecules. (springer.com)
  • Biochemical Studies on Sphingolipids of Artemia franciscana: Novel Neutral Glycosphingolipids │ J. Lipid Res. (ritsumei.ac.jp)
  • It has been reported that Aβ generation and accumulation occur in membrane microdomains, called lipid rafts, which are enriched in cholesterol and glycosphingolipids. (elsevierpure.com)
  • Glycosphingolipids are a subtype of glycolipids containing the amino alcohol sphingosine. (wikipedia.org)
  • Glycosphingolipids (GSLs) are a subclass of glycolipids that are found in cell membranes of various organisms ranging from bacteria to humans. (springer.com)
  • Fatty acids called glycosphingolipids prevent infection from developing within the GI system. (isitbadforyou.com)
  • Deficiency or absence of alpha-galactosidase A (α-GAL A) activity as a result of gene mutations in the GLA gene (Xq21.3-q22) leads to lysosomal accumulation of neutral glycosphingolipids, most notably globotriaosylceramide (G3b). (medscape.com)
  • As a consequence of the αGal A deficiency, neutral glycosphingolipids, mainly globotriaosylceramide (Gb3), accumulate in a variety of cells and tissues, leading to a wide clinical spectrum of clinical manifestations [ 4 ]. (biomedcentral.com)
  • In general, glycosphingolipids can be categorized into two groups: neutral glycosphingolipids (also called glycosphingolipids) and negatively charged glycosphingolipids. (wikipedia.org)
  • Glycosphingolipid deposits in lysosomes of endothelial, perithelial, and smooth muscle cells of blood vessels cause swelling into the blood vessel lumen. (medscape.com)
  • Angiokeratoma corporis diffusum is the cutaneous hallmark of Fabry disease, an X-linked inherited disorder caused by a deficiency in the lysosomal enzyme alpha-galactosidase A. Decreased or absent enzyme activity causes uncleaved glycosphingolipids to accumulate in various cell types, particularly in the vascular endothelium, smooth muscle cells, and pericytes, causing ischemia and infarction of tissues. (medscape.com)
  • These glycosphingolipids accumulate in many different types of cells. (medscape.com)
  • Persons with Fabry disease who have type AB or B blood also accumulate blood group B glycosphingolipids (those with alpha-galactosyl-terminated residues) and can have more severe Fabry disease (related to greater body substrate mass) than patients with blood group A. This is because these blood groups have two additional terminal alpha-galactosyl moieties. (medscape.com)
  • Glycosphingolipid binding specificities of rotavirus: identification of a sialic acid-binding epitope. (expasy.org)
  • Characterization of a Novel Rhamnose-containing Acidic Glycosphingolipid from the Ascidian Halocynthia aurantium. (ritsumei.ac.jp)
  • Structural characterization of neutral glycosphingolipids from 3T3-L1 adipocytes. (ritsumei.ac.jp)
  • Prosaposin (PSAP) modulates glycosphingolipid metabolism and variants have been linked to Parkinson's disease (PD). (nature.com)
  • Guest editor Sandro Sonnino introduces the contents of the issue in a fascinating interview conducted by Ioannis Tsagakis, starting with eight Review articles that focus on the role of glycosphingolipids in diseases as diverse as Parkinson's disease, cystic fibrosis, infertility, and parasite infections. (febs.org)
  • Structural analysis of neutral glycosphingolipids from the silkworm Bombyx mori and the difference in ceramide composition between larvae and pupae. (ritsumei.ac.jp)
  • Glycosphingolipids were once considered to play a purely structural role in cell membranes, but it has since become apparent that they play a key role in the nervous system and are associated with several diseases. (febs.org)
  • Neprilysin (NEP), a neutral endopeptidase, is one of the major Aβ-degrading enzymes in the brain. (elsevierpure.com)
  • Deposition of glycosphingolipids can be attributed to both endogenous production and diffusion of material from the circulation. (medscape.com)
  • Extended globo-series glycosphingolipids that mediate cell adhesion and signaling. (or.jp)
  • Glycosphingolipids play also important role in oncogenesis and ontogenesis. (wikipedia.org)
  • In this review, strategies to synthesize these complex glycosphingolipids are presented. (springer.com)
  • While most studies use Gb 3 extracts, chemical synthesis provides a unique tool to access different tailor-made Gb 3 glycosphingolipids. (springer.com)
  • Gb 3 glycosphingolipids are the specific receptors for bacterial Shiga toxin. (springer.com)
  • neutral pH for daily use. (lepaniervertqc.com)
  • Earth science ceramide care shampoo, volumizing is a color-safe, gentle shampoo with neutral ph for daily use. (zafra.com)
  • 19. Isolation and characterization of the major acidic glycosphingolipids from the liver of the English sole (Parophrys vetulus). (nih.gov)
  • Isolation and characterization of the major glycosphingolipids from the liver of the rainbow trout (Oncorhynchus mykiss): identification of an abundant source of 9-O-acetyl GD3. (nih.gov)
  • Deficiency or absence of alpha-galactosidase A (α-GAL A) activity as a result of gene mutations in the GLA gene (Xq21.3-q22) leads to lysosomal accumulation of neutral glycosphingolipids, most notably globotriaosylceramide (G3b). (medscape.com)
  • The progressive endothelial accumulation of glycosphingolipids characteristic of the disease can lead to clinical abnormalities of the skin, eye, kidney, heart, brain, and peripheral nervous system. (medscape.com)
  • Diffuse, abnormal accumulation of glycosphingolipids occurs in all tissues, producing swelling and proliferation of endothelial cells. (medscape.com)
  • Fabry's disease is a rare, X-linked disorder of the glycosphingolipid metabolism, in which a partial or total deficiency of a lysosomal alpha(α)-galactosidase results in the progressive accumulation of neutral glycosphingolipids with terminal alpha galactose moieties (i.e., cerebroside di- and trihexoside) in most body fluids and tissues. (ewha.ac.kr)
  • Angiokeratoma corporis diffusum is the cutaneous hallmark of Fabry disease, an X-linked inherited disorder caused by a deficiency in the lysosomal enzyme alpha-galactosidase A. Decreased or absent enzyme activity causes uncleaved glycosphingolipids to accumulate in various cell types, particularly in the vascular endothelium, smooth muscle cells, and pericytes, causing ischemia and infarction of tissues. (medscape.com)
  • Fabry disease is an X-linked lysosomal disorder which results in excessive deposition of neutral glycosphingolipids in the vascular endothelium of multiple organs as well as in epithelial and smooth muscle cells. (medscape.com)
  • Fabry disease is an X-linked lysosomal disorder that leads to excessive deposition of neutral glycosphingolipids in the vascular endothelium of several organs and in epithelial and smooth muscle cells. (medscape.com)
  • Fabrazyme is a hydrolytic lysosomal neutral glycosphingolipid-specific enzyme indicated for the treatment of adult and pediatric patients 2 years of age and older with confirmed Fabry disease. (nih.gov)
  • Glucocerebrosidase (GCase), a lysosomal hydrolase encoded by the GBA gene, is responsible for the metabolism of the glycosphingolipid substrates glucosylceramide (GlcCer) and glucosylsphingosine (GlcSph). (biomedcentral.com)
  • 12. Isolation and structural characterization of glycosphingolipids of in vitro propagated bovine aortic endothelial cells. (nih.gov)
  • Glycosphingolipid deposits in lysosomes of endothelial, perithelial, and smooth muscle cells of blood vessels cause swelling into the blood vessel lumen. (medscape.com)
  • Inherited defects in the degradation of glycosphingolipids (GSLs) cause a group of severe diseases known as GSL storage disorders. (nih.gov)
  • Previously, it was found that a novel class of neutral fucosylated glycosphingolipids (GSLs) is required for male fertility. (ox.ac.uk)
  • Increasing levels of neutral complex VLC-PUFA GSLs also correlated with the progression of spermatogenesis in a series of male sterile mutants with arrests at different stages of spermatogenesis. (ox.ac.uk)
  • Abnormalities in glycosphingolipid (GSL) biosynthesis have been implicated in the oncogenesis and malignancy of brain tumours. (ox.ac.uk)
  • Deposition of glycosphingolipids can be attributed to both endogenous production and diffusion of material from the circulation. (medscape.com)
  • The type IIcytokeratins consist of basic or neutral proteins which are arranged in pairs of heterotypic keratinchains coexpressed during differentiation of simple and stratified epithelial tissues. (tech-strategy.org)
  • A subclass of GLYCOSPHINGOLIPIDS containing one or more sugars within their head group connected directly to a ceramide moiety. (nih.gov)
  • Simplifying complexity: genetically resculpting glycosphingolipid synthesis pathways in mice to reveal function. (nih.gov)
  • Glycosphingolipids play also important role in oncogenesis and ontogenesis. (wikipedia.org)
  • The carbohydrate structures of the major glycosphingolipids from the liver of the rainbow trout Oncorhynchus mykiss have been examined. (nih.gov)
  • 7. Characterization of glycosphingolipids from gastrointestinal stromal tumours. (nih.gov)
  • Persons with Fabry disease who have type AB or B blood also accumulate blood group B glycosphingolipids (those with alpha-galactosyl-terminated residues) and can have more severe Fabry disease (related to greater body substrate mass) than patients with blood group A. This is because these blood groups have two additional terminal alpha-galactosyl moieties. (medscape.com)
  • The selective uptake of PEGOL-60 in the hurt brain regions of CP animals could be explained because of its ability (i) to cross the impaired BBB, (ii) to diffuse efficiently within the brain parenchyma due to its neutral charge, and Carglumic Acid (iii) to be picked up by phagocytic activated Mi/Ma. (thetechnoant.info)
  • Glycosphingolipid binding specificities of rotavirus: identification of a sialic acid-binding epitope. (expasy.org)
  • The strategy involves orthogonal techniques: total N-glycan profiling and quantitative analysis of neutral monosaccharides and sialic acids, where a mAb is used as a model glycoprotein and this workflow can be applied throughout a biopharmaceutical's product life cycle. (ludger.com)
  • There is a considerable variation in this group of glycosphingolipids. (gerli.com)
  • Your order is certified plastic negative, and it will ship carbon neutral with offsets we purchase to help combat climate change. (beauty-heroes.com)
  • In general, glycosphingolipids can be categorized into two groups: neutral glycosphingolipids (also called glycosphingolipids) and negatively charged glycosphingolipids. (wikipedia.org)
  • The separation of glycosphingolipids into groups and subgroups is made according to the structure of the carbohydrate moiety but they show also great variations in the acyl moiety as well as in the sphingosine moiety. (gerli.com)
  • 17. Analysis of glycosphingolipids of human head and neck carcinomas with comparison to normal tissue. (nih.gov)
  • These glycosphingolipids accumulate in many different types of cells. (medscape.com)
  • ProSight PTM is normally a tool for using the neutral mass data inferred from mass spectra to identify and characterize proteins. (cancerdir.com)
  • Neutral glycosphingolipids are also important, for example as blood type antigens. (wikipedia.org)
  • An extensive review of these glycosphingolipids found in bacteria, fungi, plants, and animals was written by Tan RX et al. (gerli.com)
  • Fiedler K, Kobayashi download, Kurzchalia TV, Simons K. Glycosphingolipid-enriched, free cascades in control signaling in ARM phosphatases. (erik-mill.de)
  • 9. Comparison of the glycosphingolipids of human-induced pluripotent stem cells and human embryonic stem cells. (nih.gov)
  • Another, more polar, neutral glycolipid appeared exclusively after stimulation of responsive B cells. (trnds.co)
  • Footnotes Publisher's Take note Springer Nature continues to be neutral in regards to to jurisdictional promises in released maps and institutional affiliations. (thetechnoant.info)