G(M1) Ganglioside: A specific monosialoganglioside that accumulates abnormally within the nervous system due to a deficiency of GM1-b-galactosidase, resulting in GM1 gangliosidosis.G(M3) Ganglioside: 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.G(M2) Ganglioside: 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.Gangliosides: 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, Thin Layer: 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)Sialyltransferases: A group of enzymes with the general formula CMP-N-acetylneuraminate:acceptor N-acetylneuraminyl transferase. They catalyze the transfer of N-acetylneuraminic acid from CMP-N-acetylneuraminic acid to an acceptor, which is usually the terminal sugar residue of an oligosaccharide, a glycoprotein, or a glycolipid. EC 2.4.99.-.Glycosphingolipids: 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)G(M2) Activator Protein: An essential cofactor for the degradation of G(M2)GANGLIOSIDE by lysosomal BETA-N-ACETYLHEXOSAMINIDASES. Genetic mutations resulting in loss of G(M2) activator protein are one of the causes of TAY-SACHS DISEASE, AB VARIANT.Carbohydrate Sequence: The sequence of carbohydrates within POLYSACCHARIDES; GLYCOPROTEINS; and GLYCOLIPIDS.Neuraminidase: An enzyme that catalyzes the hydrolysis of alpha-2,3, alpha-2,6-, and alpha-2,8-glycosidic linkages (at a decreasing rate, respectively) of terminal sialic residues in oligosaccharides, glycoproteins, glycolipids, colominic acid, and synthetic substrate. (From Enzyme Nomenclature, 1992)Cholera Toxin: An ENTEROTOXIN from VIBRIO CHOLERAE. It consists of two major protomers, the heavy (H) or A subunit and the B protomer which consists of 5 light (L) or B subunits. The catalytic A subunit is proteolytically cleaved into fragments A1 and A2. The A1 fragment is a MONO(ADP-RIBOSE) TRANSFERASE. The B protomer binds cholera toxin to intestinal epithelial cells, and facilitates the uptake of the A1 fragment. The A1 catalyzed transfer of ADP-RIBOSE to the alpha subunits of heterotrimeric G PROTEINS activates the production of CYCLIC AMP. Increased levels of cyclic AMP are thought to modulate release of fluid and electrolytes from intestinal crypt cells.Sialic Acids: A group of naturally occurring N-and O-acyl derivatives of the deoxyamino sugar neuraminic acid. They are ubiquitously distributed in many tissues.Sandhoff Disease: An autosomal recessive neurodegenerative disorder characterized by an accumulation of G(M2) GANGLIOSIDE in neurons and other tissues. It is caused by mutation in the common beta subunit of HEXOSAMINIDASE A and HEXOSAMINIDASE B. Thus this disease is also known as the O variant since both hexosaminidase A and B are missing. Clinically, it is indistinguishable from TAY-SACHS DISEASE.Tay-Sachs Disease: An autosomal recessive neurodegenerative disorder characterized by the onset in infancy of an exaggerated startle response, followed by paralysis, dementia, and blindness. It is caused by mutation in the alpha subunit of the HEXOSAMINIDASE A resulting in lipid-laden ganglion cells. It is also known as the B variant (with increased HEXOSAMINIDASE B but absence of hexosaminidase A) and is strongly associated with Ashkenazic Jewish ancestry.Hexosaminidase A: A mammalian beta-hexosaminidase isoform that is a heteromeric protein comprized of both hexosaminidase alpha and hexosaminidase beta subunits. Deficiency of hexosaminidase A due to mutations in the gene encoding the hexosaminidase alpha subunit is a case of TAY-SACHS DISEASE. Deficiency of hexosaminidase A and HEXOSAMINIDASE B due to mutations in the gene encoding the hexosaminidase beta subunit is a case of SANDHOFF DISEASE.beta-N-Acetylhexosaminidases: A hexosaminidase specific for non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides. It acts on GLUCOSIDES; GALACTOSIDES; and several OLIGOSACCHARIDES. Two specific mammalian isoenzymes of beta-N-acetylhexoaminidase are referred to as HEXOSAMINIDASE A and HEXOSAMINIDASE B. Deficiency of the type A isoenzyme causes TAY-SACHS DISEASE, while deficiency of both A and B isozymes causes SANDHOFF DISEASE. The enzyme has also been used as a tumor marker to distinguish between malignant and benign disease.Gangliosidoses: A group of autosomal recessive lysosomal storage disorders marked by the accumulation of GANGLIOSIDES. They are caused by impaired enzymes or defective cofactors required for normal ganglioside degradation in the LYSOSOMES. Gangliosidoses are classified by the specific ganglioside accumulated in the defective degradation pathway.Sphingolipid Activator Proteins: 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.Glycolipids: 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)Neuraminic AcidsHexosaminidase B: A mammalian beta-hexosaminidase isoform that is comprized of hexosaminidase beta subunits. Deficiency of hexosaminidase B due to mutations in the gene encoding the hexosaminidase beta subunit is a case of SANDHOFF DISEASE.N-Acetylneuraminic Acid: An N-acyl derivative of neuraminic acid. N-acetylneuraminic acid occurs in many polysaccharides, glycoproteins, and glycolipids in animals and bacteria. (From Dorland, 28th ed, p1518)Gangliosidosis, GM1: An autosomal recessive neurodegenerative disorder caused by the absence or deficiency of BETA-GALACTOSIDASE. It is characterized by intralysosomal accumulation of G(M1) GANGLIOSIDE and oligosaccharides, primarily in neurons of the central nervous system. The infantile form is characterized by MUSCLE HYPOTONIA, poor psychomotor development, HIRSUTISM, hepatosplenomegaly, and facial abnormalities. The juvenile form features HYPERACUSIS; SEIZURES; and psychomotor retardation. The adult form features progressive DEMENTIA; ATAXIA; and MUSCLE SPASTICITY. (From Menkes, Textbook of Child Neurology, 5th ed, pp96-7)Spectrometry, Mass, Fast Atom Bombardment: 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.Lactosylceramides: 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.Gangliosidoses, GM2: A group of recessively inherited diseases characterized by the intralysosomal accumulation of G(M2) GANGLIOSIDE in the neuronal cells. Subtypes include mutations of enzymes in the BETA-N-ACETYLHEXOSAMINIDASES system or G(M2) ACTIVATOR PROTEIN leading to disruption of normal degradation of GANGLIOSIDES, a subclass of ACIDIC GLYCOSPHINGOLIPIDS.Globosides: 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.N-Acetylgalactosaminyltransferases: Enzymes that catalyze the transfer of N-acetylgalactosamine from a nucleoside diphosphate N-acetylgalactosamine to an acceptor molecule which is frequently another carbohydrate. EC 2.4.1.-.Carbohydrate Conformation: The characteristic 3-dimensional shape of a carbohydrate.Saposins: A group of four homologous sphingolipid activator proteins that are formed from proteolytic cleavage of a common protein precursor molecule referred to as prosaposin.Guillain-Barre Syndrome: An acute inflammatory autoimmune neuritis caused by T cell- mediated cellular immune response directed towards peripheral myelin. Demyelination occurs in peripheral nerves and nerve roots. The process is often preceded by a viral or bacterial infection, surgery, immunization, lymphoma, or exposure to toxins. Common clinical manifestations include progressive weakness, loss of sensation, and loss of deep tendon reflexes. Weakness of respiratory muscles and autonomic dysfunction may occur. (From Adams et al., Principles of Neurology, 6th ed, pp1312-1314)Ceramides: 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.Lipidoses: Conditions characterized by abnormal lipid deposition due to disturbance in lipid metabolism, such as hereditary diseases involving lysosomal enzymes required for lipid breakdown. They are classified either by the enzyme defect or by the type of lipid involved.Membrane Microdomains: Detergent-insoluble CELL MEMBRANE components. They are enriched in SPHINGOLIPIDS and CHOLESTEROL and clustered with glycosyl-phosphatidylinositol (GPI)-anchored proteins.Brain Chemistry: 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.Tetanus Toxin: Protein synthesized by CLOSTRIDIUM TETANI as a single chain of ~150 kDa with 35% sequence identity to BOTULINUM TOXIN that is cleaved to a light and a heavy chain that are linked by a single disulfide bond. Tetanolysin is the hemolytic and tetanospasmin is the neurotoxic principle. The toxin causes disruption of the inhibitory mechanisms of the CNS, thus permitting uncontrolled nervous activity, leading to fatal CONVULSIONS.Miller Fisher Syndrome: A variant of the GUILLAIN-BARRE SYNDROME characterized by the acute onset of oculomotor dysfunction, ataxia, and loss of deep tendon reflexes with relative sparing of strength in the extremities and trunk. The ataxia is produced by peripheral sensory nerve dysfunction and not by cerebellar injury. Facial weakness and sensory loss may also occur. The process is mediated by autoantibodies directed against a component of myelin found in peripheral nerves. (Adams et al., Principles of Neurology, 6th ed, p1313; Neurology 1987 Sep;37(9):1493-8)Galactosyltransferases: Enzymes that catalyze the transfer of galactose from a nucleoside diphosphate galactose to an acceptor molecule which is frequently another carbohydrate. EC 2.4.1.-.Sulfoglycosphingolipids: GLYCOSPHINGOLIPIDS with a sulfate group esterified to one of the sugar groups.Cerebrosides: 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)Ganglioside Galactosyltransferase: Catalyzes the final step in the galactocerebroside biosynthesis pathway.Starfish: Echinoderms having bodies of usually five radially disposed arms coalescing at the center.Hexosaminidases: Enzymes that catalyze the hydrolysis of N-acylhexosamine residues in N-acylhexosamides. Hexosaminidases also act on GLUCOSIDES; GALACTOSIDES; and several OLIGOSACCHARIDES.Carbohydrates: 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.Molecular Sequence Data: 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.Antibodies, Monoclonal: Antibodies produced by a single clone of cells.Galactose Oxidase: An enzyme that oxidizes galactose in the presence of molecular oxygen to D-galacto-hexodialdose. It is a copper protein. EC 126.96.36.199.Cell Membrane: The lipid- and protein-containing, selectively permeable membrane that surrounds the cytoplasm in prokaryotic and eukaryotic cells.Membrane Lipids: Lipids, predominantly phospholipids, cholesterol and small amounts of glycolipids found in membranes including cellular and intracellular membranes. These lipids may be arranged in bilayers in the membranes with integral proteins between the layers and peripheral proteins attached to the outside. Membrane lipids are required for active transport, several enzymatic activities and membrane formation.Polyradiculoneuropathy: Diseases characterized by injury or dysfunction involving multiple peripheral nerves and nerve roots. The process may primarily affect myelin or nerve axons. Two of the more common demyelinating forms are acute inflammatory polyradiculopathy (GUILLAIN-BARRE SYNDROME) and POLYRADICULONEUROPATHY, CHRONIC INFLAMMATORY DEMYELINATING. Polyradiculoneuritis refers to inflammation of multiple peripheral nerves and spinal nerve roots.Monoglycerides: GLYCEROL esterified with a single acyl (FATTY ACIDS) chain.Molecular Mimicry: The structure of one molecule that imitates or simulates the structure of a different molecule.Toxins, Biological: Specific, characterizable, poisonous chemicals, often PROTEINS, with specific biological properties, including immunogenicity, produced by microbes, higher plants (PLANTS, TOXIC), or ANIMALS.beta-Hexosaminidase beta Chain: The beta subunit of hexosaminidase A and hexosamininidase B. Mutations in the gene that encodes this protein are linked to SANDHOFF DISEASE and can result in loss of both hexosaminidase A activity and hexosaminidase B activity.Hymecromone: A coumarin derivative possessing properties as a spasmolytic, choleretic and light-protective agent. It is also used in ANALYTICAL CHEMISTRY TECHNIQUES for the determination of NITRIC ACID.Neuroblastoma: A common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death. This tumor is the most common intraabdominal malignancy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system. Histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa. Neuroblastomas may be associated with the opsoclonus-myoclonus syndrome. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2099-2101; Curr Opin Oncol 1998 Jan;10(1):43-51)Campylobacter jejuni: A species of bacteria that resemble small tightly coiled spirals. Its organisms are known to cause abortion in sheep and fever and enteritis in man and may be associated with enteric diseases of calves, lambs, and other animals.Melanoma: A malignant neoplasm derived from cells that are capable of forming melanin, which may occur in the skin of any part of the body, in the eye, or, rarely, in the mucous membranes of the genitalia, anus, oral cavity, or other sites. It occurs mostly in adults and may originate de novo or from a pigmented nevus or malignant lentigo. Melanomas frequently metastasize widely, and the regional lymph nodes, liver, lungs, and brain are likely to be involved. The incidence of malignant skin melanomas is rising rapidly in all parts of the world. (Stedman, 25th ed; from Rook et al., Textbook of Dermatology, 4th ed, p2445)Acetylgalactosamine: The N-acetyl derivative of galactosamine.Enterotoxins: Substances that are toxic to the intestinal tract causing vomiting, diarrhea, etc.; most common enterotoxins are produced by bacteria.Mass Spectrometry: An analytical method used in determining the identity of a chemical based on its mass using mass analyzers/mass spectrometers.Glucosylceramides: 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.Sphingosine: 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)Vibrio cholerae: The etiologic agent of CHOLERA.Sphingolipids: 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)Octoxynol: Nonionic surfactant mixtures varying in the number of repeating ethoxy (oxy-1,2-ethanediyl) groups. They are used as detergents, emulsifiers, wetting agents, defoaming agents, etc. Octoxynol-9, the compound with 9 repeating ethoxy groups, is a spermatocide.Magnetic Resonance Spectroscopy: 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).Brain: The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.Sphingomyelins: A class of sphingolipids found largely in the brain and other nervous tissue. They contain phosphocholine or phosphoethanolamine as their polar head group so therefore are the only sphingolipids classified as PHOSPHOLIPIDS.Cell Line: Established cell cultures that have the potential to propagate indefinitely.Toxoids: Preparations of pathogenic organisms or their derivatives made nontoxic and intended for active immunologic prophylaxis. They include deactivated toxins. Anatoxin toxoids are distinct from anatoxins that are TROPANES found in CYANOBACTERIA.Liposomes: Artificial, single or multilaminar vesicles (made from lecithins or other lipids) that are used for the delivery of a variety of biological molecules or molecular complexes to cells, for example, drug delivery and gene transfer. They are also used to study membranes and membrane proteins.Detergents: Purifying or cleansing agents, usually salts of long-chain aliphatic bases or acids, that exert cleansing (oil-dissolving) and antimicrobial effects through a surface action that depends on possessing both hydrophilic and hydrophobic properties.Micelles: Particles consisting of aggregates of molecules held loosely together by secondary bonds. The surface of micelles are usually comprised of amphiphatic compounds that are oriented in a way that minimizes the energy of interaction between the micelle and its environment. Liquids that contain large numbers of suspended micelles are referred to as EMULSIONS.Tumor Cells, Cultured: Cells grown in vitro from neoplastic tissue. If they can be established as a TUMOR CELL LINE, they can be propagated in cell culture indefinitely.Cattle: 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.Galactose: An aldohexose that occurs naturally in the D-form in lactose, cerebrosides, gangliosides, and mucoproteins. Deficiency of galactosyl-1-phosphate uridyltransferase (GALACTOSE-1-PHOSPHATE URIDYL-TRANSFERASE DEFICIENCY DISEASE) causes an error in galactose metabolism called GALACTOSEMIA, resulting in elevations of galactose in the blood.Botulinum Toxins: Toxic proteins produced from the species CLOSTRIDIUM BOTULINUM. The toxins are synthesized as a single peptide chain which is processed into a mature protein consisting of a heavy chain and light chain joined via a disulfide bond. The botulinum toxin light chain is a zinc-dependent protease which is released from the heavy chain upon ENDOCYTOSIS into PRESYNAPTIC NERVE ENDINGS. Once inside the cell the botulinum toxin light chain cleaves specific SNARE proteins which are essential for secretion of ACETYLCHOLINE by SYNAPTIC VESICLES. This inhibition of acetylcholine release results in muscular PARALYSIS.Glycosyltransferases: Enzymes that catalyze the transfer of glycosyl groups to an acceptor. Most often another carbohydrate molecule acts as an acceptor, but inorganic phosphate can also act as an acceptor, such as in the case of PHOSPHORYLASES. Some of the enzymes in this group also catalyze hydrolysis, which can be regarded as transfer of a glycosyl group from the donor to water. Subclasses include the HEXOSYLTRANSFERASES; PENTOSYLTRANSFERASES; SIALYLTRANSFERASES; and those transferring other glycosyl groups. EC 2.4.Kinetics: The rate dynamics in chemical or physical systems.Cells, Cultured: Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.Lysosomes: A class of morphologically heterogeneous cytoplasmic particles in animal and plant tissues characterized by their content of hydrolytic enzymes and the structure-linked latency of these enzymes. The intracellular functions of lysosomes depend on their lytic potential. The single unit membrane of the lysosome acts as a barrier between the enzymes enclosed in the lysosome and the external substrate. The activity of the enzymes contained in lysosomes is limited or nil unless the vesicle in which they are enclosed is ruptured. Such rupture is supposed to be under metabolic (hormonal) control. (From Rieger et al., Glossary of Genetics: Classical and Molecular, 5th ed)GalactosamineAntibody Specificity: The property of antibodies which enables them to react with some ANTIGENIC DETERMINANTS and not with others. Specificity is dependent on chemical composition, physical forces, and molecular structure at the binding site.Cytidine Monophosphate N-Acetylneuraminic Acid: A nucleoside monophosphate sugar which donates N-acetylneuraminic acid to the terminal sugar of a ganglioside or glycoprotein.Oligosaccharides: Carbohydrates consisting of between two (DISACCHARIDES) and ten MONOSACCHARIDES connected by either an alpha- or beta-glycosidic link. They are found throughout nature in both the free and bound form.Hydrolysis: The process of cleaving a chemical compound by the addition of a molecule of water.Bacterial Toxins: Toxic substances formed in or elaborated by bacteria; they are usually proteins with high molecular weight and antigenicity; some are used as antibiotics and some to skin test for the presence of or susceptibility to certain diseases.Neutral Glycosphingolipids: 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.Golgi Apparatus: A stack of flattened vesicles that functions in posttranslational processing and sorting of proteins, receiving them from the rough ENDOPLASMIC RETICULUM and directing them to secretory vesicles, LYSOSOMES, or the CELL MEMBRANE. The movement of proteins takes place by transfer vesicles that bud off from the rough endoplasmic reticulum or Golgi apparatus and fuse with the Golgi, lysosomes or cell membrane. (From Glick, Glossary of Biochemistry and Molecular Biology, 1990)Amish: An ethnic group with shared religious beliefs. Originating in Switzerland in the late 1600s, and first migrating to the mid-Atlantic, they now live throughout Eastern and Mid-Western United States and elsewhere. Communities are usually close-knit and marriage is within the community.Neurites: In tissue culture, hairlike projections of neurons stimulated by growth factors and other molecules. These projections may go on to form a branched tree of dendrites or a single axon or they may be reabsorbed at a later stage of development. "Neurite" may refer to any filamentous or pointed outgrowth of an embryonal or tissue-culture neural cell.Filipin: A complex of polyene antibiotics obtained from Streptomyces filipinensis. Filipin III alters membrane function by interfering with membrane sterols, inhibits mitochondrial respiration, and is proposed as an antifungal agent. Filipins I, II, and IV are less important.Protein Binding: The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.Liver: A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.Cerebellum: The part of brain that lies behind the BRAIN STEM in the posterior base of skull (CRANIAL FOSSA, POSTERIOR). It is also known as the "little brain" with convolutions similar to those of CEREBRAL CORTEX, inner white matter, and deep cerebellar nuclei. Its function is to coordinate voluntary movements, maintain balance, and learn motor skills.Gas Chromatography-Mass Spectrometry: A microanalytical technique combining mass spectrometry and gas chromatography for the qualitative as well as quantitative determinations of compounds.Neurons: The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the NERVOUS SYSTEM.Glucosyltransferases: 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.-.beta-Cyclodextrins: Cyclic GLUCANS consisting of seven (7) glucopyranose units linked by 1,4-glycosidic bonds.Fatty Acids: 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)Enzyme-Linked Immunosorbent Assay: An immunoassay utilizing an antibody labeled with an enzyme marker such as horseradish peroxidase. While either the enzyme or the antibody is bound to an immunosorbent substrate, they both retain their biologic activity; the change in enzyme activity as a result of the enzyme-antibody-antigen reaction is proportional to the concentration of the antigen and can be measured spectrophotometrically or with the naked eye. Many variations of the method have been developed.Sphingolipidoses: A group of inherited metabolic disorders characterized by the intralysosomal accumulation of SPHINGOLIPIDS primarily in the CENTRAL NERVOUS SYSTEM and to a variable degree in the visceral organs. They are classified by the enzyme defect in the degradation pathway and the substrate accumulation (or storage). Clinical features vary in subtypes but neurodegeneration is a common sign.beta-Hexosaminidase alpha Chain: The alpha subunit of hexosaminidase A. Mutations in the gene that encodes this protein can result in loss of hexosaminidase A activity and are linked to TAY-SACHS DISEASE.HexosaminesChromatography, High Pressure Liquid: Liquid chromatographic techniques which feature high inlet pressures, high sensitivity, and high speed.1-Deoxynojirimycin: An alpha-glucosidase inhibitor with antiviral action. Derivatives of deoxynojirimycin may have anti-HIV activity.Molecular Structure: The location of the atoms, groups or ions relative to one another in a molecule, as well as the number, type and location of covalent bonds.
GM1 gangliosides are found in lipid rafts on the cell surface. B subunit complexes labelled with fluorescent tags or ... Enterotoxin Ganglioside Ryan KJ; Ray CG, eds. (2004). Sherris Medical Microbiology (4th ed.). McGraw Hill. p. 375. ISBN 0-8385- ... Cholera toxin acts by the following mechanism: First, the B subunit ring of the cholera toxin binds to GM1 gangliosides on the ...
... is a ganglioside. It is also known as CD77, Gb3, and ceramide trihexoside. It is one of the few clusters ...
... uridine diphosphoacetylgalactosamine-ganglioside GM3 acetylgalactosaminyltransferase, ganglioside GM2 synthase, ganglioside GM3 ... Dicesare, J.L.; Dain, J.A. (1971). "The enzymic synthesis of ganglioside. IV. UDP-N-acetylgalactosamine: (N-acetylneuraminyl)- ... "Interruption of ganglioside synthesis produces central nervous system degeneration and altered axon-glial interactions". Proc. ... ceramide This enzyme catalyses the formation of the gangliosides (i.e. sialic-acid-containing glycosphingolipids) GM2, GD2 and ...
Anti-ganglioside antibodies "Mitumomab - AdisInsight". Adisinsight.springer.com. 2005-06-13. Retrieved 2017-01-06. Clinical ...
Antibodies to ganglioside are found to be elevated in coeliac disease. Recent studies show that gliadin can cross-link to ... IgA to gangliosides have been observed in Guillain-Barré syndrome. IgM. IgM antibodies have been detected in early work, but ... Autoantigenic gangliosides that are currently known are GD3, GM1, GQ3 and GT1. Anti-GD3 antibodies have been found in ... 1994). "IgM ganglioside GM1 antibodies in patients with autoimmune disease or neuropathy, and controls". J. Clin. Pathol. 47 (4 ...
... (monosialotetrahexosylganglioside) the "prototype" ganglioside, is a member of the ganglio series of gangliosides which ... Sphingolipidoses Structures of GM1, GM2, GM3 gangliosides Mocchetti I (2005). "Exogenous gangliosides, neuronal plasticity and ... The secreted toxin attaches to the surface of the host mucosa cell by binding to GM1 gangliosides. GM1 consists of a sialic ... 1994). "IgM ganglioside GM1 antibodies in patients with autoimmune disease or neuropathy, and controls". J. Clin. Pathol. 47 (4 ...
... (monosialodihexosylganglioside) is a type of ganglioside. The letter G refers to ganglioside, and M is for monosialic acid ... The numbering is based on its relative mobility in electrophoresis among other monosialic gangliosides. Its structure can be ...
Gangliosides are made and biodegraded rapidly in early life as the brain develops. Except in some rare, late-onset forms, the ... GM2 (ganglioside) Mahuran DJ (1999-10-08). "Biochemical consequences of mutations causing the GM2 gangliosidoses". Biochimica ... This enzyme catalyzes the biodegradation of fatty acid derivatives known as gangliosides. The diseases are better known by ... The disease occurs when harmful quantities of cell membrane gangliosides accumulate in the brain's nerve cells, eventually ...
The ganglioside-converting enzyme plasma membrane ganglioside sialidase (PMGS), which is involved in the activation of TrkA at ... Da Silva, JS; Hasegawa, T; Miyagi, T; Dotti, CG; Abad-Rodriguez, J (May 2005). "Asymmetric membrane ganglioside sialidase ...
The enzyme hydrolyzes monomerically dispersed GM1 ganglioside molecules". J Biol Chem. 279 (5): 3169-3179. doi:10.1074/jbc. ...
The hydrolysis of GM2-ganglioside requires three proteins. Two of them are subunits of hexosaminidase A; the third is a small ... This red spot is a retinal area that appears red because of gangliosides in the surrounding retinal ganglion cells. The ... Deficiency in any one of these proteins leads to ganglioside storage, primarily in the lysosomes of neurons. Tay-Sachs disease ... Igdoura SA, Mertineit C, Trasler JM, Gravel RA (1999). "Sialidase-mediated depletion of GM2 ganglioside in Tay-Sachs neuroglia ...
Separation of membranes with different ratios of ganglioside sialylating activity to gangliosides". Biochem. J. 168 (3): 325-32 ... Landa CA, Maccioni HJ, Arce A, Caputto R (1977). "The biosynthesis of brain gangliosides. ...
Lumbar puncture and serum test for anti-ganglioside antibodies. These antibodies are present in the branch of CIDP diseases ... A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol 24: 73-78 Nobile-Orazio E. Multifocal ... in 1988 described a neuropathy which was later found to be associated with IgM autoantibodies directed against GM1 gangliosides ...
Ganglioside-induced differentiation-associated protein 1 is a protein that in humans is encoded by the GDAP1 gene. This gene ... "Entrez Gene: GDAP1 ganglioside-induced differentiation-associated protein 1". Liu H, Nakagawa T, Kanematsu T, et al. (1999). " ... 2002). "Ganglioside-induced differentiation-associated protein-1 is mutant in Charcot-Marie-Tooth disease type 4A/8q21". Nat. ... 2002). "The gene encoding ganglioside-induced differentiation-associated protein 1 is mutated in axonal Charcot-Marie-Tooth ...
Role of ganglioside metabolism in the pathogenesis of Alzheimer's disease - a review-JOURNAL OF LIPID RESEARCH Volume: 49 Issue ... Gangliosides: the most complex animal glycolipids. They contain negatively charged oligosacchrides with one or more sialic acid ... They may be as complicated a set of compounds as the negatively charged gangliosides in animals. Glycophosphatidylinositols: a ... residues; more than 200 different gangliosides have been identified. They are most abundant in nerve cells. Globosides: ...
... and reduced the GM2 ganglioside storage in the parenchyma. HEXB has been found to interact with HEXA and ganglioside. GRCh38: ... characterized by the accumulation of GM2 ganglioside. Genetic defects in HEXB can result in the accumulation of GM2 ganglioside ... Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative ... Bolhuis PA, Oonk JG, Kamp PE, Ris AJ, Michalski JC, Overdijk B, Reuser AJ (January 1987). "Ganglioside storage, hexosaminidase ...
It is localized in the plasma membrane, and its activity is specific for gangliosides. It may play a role in modulating the ... Wang Y, Yamaguchi K, Wada T, Hata K, Zhao X, Fujimoto T, Miyagi T (2002). "A close association of the ganglioside-specific ... Ha KT, Lee YC, Cho SH, Kim JK, Kim CH (2004). "Molecular characterization of membrane type and ganglioside-specific sialidase ( ... Yamaguchi K, Hata K, Wada T, Moriya S, Miyagi T (2006). "Epidermal growth factor-induced mobilization of a ganglioside-specific ...
Antibodies to these gangliosides have been found elevated in coeliac disease A sizable fraction of individuals who have gluten ... 2006). "Anti-ganglioside antibodies in coeliac disease with neurological disorders". Dig Liver Dis. 38 (3): 183-7. doi:10.1016/ ... Gulliane-Barre syndrome is associated with peripheral neuropathies, and it has been found that anti-ganglioside autoantibodies ...
Certain aspects of lipid rafts inhibit EGF receptor function: the ganglioside component of lipid rafts was shown to inhibit ... For example, fluorophores conjugated to cholera-toxin B-subunit, which binds to the raft constituent ganglioside GM1 is used ... Miljan, Erik A; Bremer, Eric G. (2002). "Regulation of growth factor receptors by gangliosides". Sci STKE. 160: RE15. doi: ... ganglioside GM1 located in lipid rafts and major histocompatibility (MHC) class I molecule. Binding of SV40 with MHC class I ...
GM2A (GM2 ganglioside activator) has been viewed as a member of the SAP family and has been called SAP-3 (sphingolipid ... Hiraiwa M, Soeda S, Kishimoto Y, O'Brien JS (1993). "Binding and transport of gangliosides by prosaposin". Proc. Natl. Acad. ... 1990). "The complete amino-acid sequences of human ganglioside GM2 activator protein and cerebroside sulfate activator protein ... GM1 ganglioside activator, dispersin, and nonspecific. It has been observed that this particular saposin activates many enzymes ...
The metabolism of Tay-Sachs ganglioside: catabolic studies with lysosomal enzymes from normal and Tay-Sachs brain tissue. J ... Demonstration of an alteration of ganglioside metabolism in Tay-Sachs disease. Biochem Biophys Res Commun 1969; 37: 526-531. ...
... together with GM2 ganglioside activator, catalyzes the degradation of the ganglioside GM2, and other molecules containing ... GM2 ganglioside activator also known as GM2A is a protein which in humans is encoded by the GM2A gene. The protein encoded by ... Hirabayashi Y, Li YT, Li SC (1983). "The protein activator specific for the enzymic hydrolysis of GM2 ganglioside in normal ... GM2A is a lipid transfer protein that stimulates the enzymatic processing of gangliosides, and also T-cell activation through ...
Gangliosides are lipids, components of cellular membranes, and the ganglioside GM2, implicated in Tay-Sachs disease, is ... The disease occurs when harmful quantities of a fatty acid derivative called a ganglioside accumulate in the nerve cells of the ... Igdoura SA, Mertineit C, Trasler JM, Gravel RA (1999). "Sialidase-mediated depletion of GM2 ganglioside in Tay-Sachs neuroglia ... By manipulating the brain's metabolism of GM2 gangliosides, an effective therapy could potentially be developed. One experiment ...
... ganglioside GD3 synthase, ganglioside GD3 synthetase sialyltransferase, CMP-NeuAc:LM1(alpha2-8) sialyltranferase, GD3 synthase ... Other names in common use include cytidine monophosphoacetylneuraminate-ganglioside GM3, alpha-2,8-sialyltransferase, ... and SAT-2. Eppler CM, Morre DJ, Keenan TW (1980). "Ganglioside biosynthesis in rat liver: alteration of sialyltransferase ...
Gangliosides are membrane-bound glycosphingolipids containing sialic acid. Ganglioside GD3 is known to be important for cell ... 2006). "Fundamental study of small interfering RNAs for ganglioside GD3 synthase gene as a therapeutic target of lung cancers ... 1997). "Requirement for GD3 ganglioside in CD95- and ceramide-induced apoptosis". Science. 277 (5332): 1652-5. doi:10.1126/ ... is a type II membrane protein that catalyzes the transfer of sialic acid from CMP-sialic acid to GM3 to produce gangliosides ...
Our results show that NB-DGJ is effective in reducing total brain ganglioside and GM2 content at early neonatal ages. ... significantly reduced total brain ganglioside and GM2 content in the Sandhoff disease (Hexb(-/-)) mice, but did not reduce the ... Sandhoff disease involves the CNS accumulation of ganglioside GM2 and asialo-GM2 (GA2) due to inherited defects in the beta- ... Ganglioside, Gangliosides, Glycosphingolipids, Mice, Mice, Knockout, Nerve Degeneration, Neuraminidase, Sandhoff Disease, ...
Results: Anti-ganglioside antibodies (IgG) were detected in 16 (32%) GBS patients and in 1 (3.3%) control using ELISA assay. ... Objective: This study aimed to determine serum levels of anti-ganglioside antibodies in children with Guillain-Barre syndrome ... The levels of anti-ganglioside antibodies in serum were measured by ELISA and immunoblotting methods using commercial kits. ... However, by employing immunoblotting technique, antibodies against seven gangliosides were found positive in 28 (56%) GBS ...
Mice display gangliosidosis; mice abnormally accumulate GM2 and GA2 ganglioside and serve as a model of Sandhoff disease. Learn ...
Yu RK, Usuki S, Ariga T. Ganglioside molecular mimicry and its pathological roles in Guillain-Barre syndrome and related ... Molecular mimicry, involving similar sequences of bacterial lipo-oligosaccharides and human gangliosides, are believed to ... jejuni infection is thought to induce an anti-ganglioside antibody. ... underlie autoimmune attack on axonal membranes in AMAN . Several gangliosides (GM1, GM1b, GD1a, and GalNAc-GD1a) on the ...
Biosynthesis of sialylated ganglioside mimics in the core oligosaccharide. J Biol Chem. 2002, 277 (1): 327-337. 10.1074/jbc. ...
Anti-GQ1b IgG antibody was positive, supporting the possibility of anti-ganglioside syndrome. This gentleman was treated with ... like Guillain-Barré syndrome/Miller Fisher syndrome and anti-ganglioside antibodies and 3) antibodies are very specific and ...
GUILLAIN-BARRE-SYNDROME ; IGG ANTI-GQ(1B) ANTIBODY ; CAMPYLOBACTER-JEJUNI ; OPTIC NEUROPATHY ; GANGLIOSIDE ; OPHTHALMOPLEGIA ; ...
The presence of these anti-ganglioside antibodies at low CD4 counts suggests that abnormal immunoregulation in HIV may ...
Here, the authors report a Thai patient diagnosed as MFS that had a positive test of antibodies against the ganglioside GQ1b. ... Here, the authors report a Thai patient diagnosed as MFS that had a positive test of antibodies against the ganglioside GQ1b. ...
Whilst some of the remaining patients have antibodies to other gangliosides, many patients with BE are reported to be ... Whilst some of the remaining patients have antibodies to other gangliosides, many patients with BE are reported to be ...
Gangliosides at the US National Library of Medicine Medical Subject Headings (MeSH) Overview of gangliosides at lipidlibrary.co ... Natural and semisynthetic gangliosides are considered possible therapeutics for neurodegenerative disorders. Gangliosides are ... More than 60 gangliosides are known, which differ from each other mainly in the position and number of NANA residues. It is a ... A ganglioside is a molecule composed of a glycosphingolipid (ceramide and oligosaccharide) with one or more sialic acids (e.g. ...
GM2 is a type of ganglioside. G refers to ganglioside, the M is for monosialic (as in it has one sialic acid), and 2 refers to ... Ganglioside GM2 activator protein Sphingolipidoses Structures of GM1, GM2, GM3 gangliosides Guetta E, Peleg L (2008). "Rapid ... Ganglioside GM2 at the US National Library of Medicine Medical Subject Headings (MeSH). ... the fact that it was the second monosialic ganglioside discovered. It is associated with GM2 gangliosidoses such as Tay-Sachs ...
Gangliosides, sialic acid containing glycosphingolipids, are normal components of the plasma membrane of vertebrate cells and ... Novak A, Lowden JA, Gravel YL, Wolfe LS (1979) Preparation of radiolabeled GM2 and GA2 gangliosides. J Lipid Res 678-681.Google ... Elucidation of the structure of ganglioside GM3 lactone. J Biochem 98, 1367-1373.PubMedGoogle Scholar ... Gangliosides, sialic acid containing glycosphingolipids, are normal components of the plasma membrane of vertebrate cells and ...
Gangliosides are glycosphingolipids that contain sialic acid ( N-acetyl- or N-glycolylneuraminic acid). They have elicited much ... Maccioni, H. J. F., Arce, A., and Caputto, R., 1971a, The biosynthesis of gangliosides, labelling of rat brain gangliosides in ... Watanabe, K., and Hakomori, S.-I., 1979, Ganglioside of human erythrocytes. A novel ganglio-side with a unique N- ... Wiegandt, H., and Schulze, B., 1969, Spleen gangliosides: the structure of ganglioside Glntet1, Z. Naturforsch. Teil B 24: 945- ...
Brain gangliosides of GM3S−/− mice. Gangliosides were isolated from brains of GM3S+/+ and GM3S−/− mice. The samples were either ... 2). Brain gangliosides from GM3S+/+ and GM3S−/− mice were treated with V. cholera sialidase. On complex brain gangliosides, the ... 1A). The GM3S−/− mice expressed major ganglioside species that comigrated with GM1b and GD1α gangliosides of the 0-series (Figs ... Ganglioside Analysis.. Gangliosides were isolated as described (9). For sialidase treatment, the reaction mixture contained the ...
Ganglioside Structure, Function and Biomedical Potential which was held at Parksville, Vancouver Island, B. C. , Canada on ... Ganglioside Structure, Function, and Biomedical Potential. Editors. * Robert Ledeen Series Title. Advances in Experimental ... Immunological Expression of Gangliosides in Multiple Sclerosis and in a Demyelinating Model Disease in Rabbits ... The Labeling of the Retina and Optic Tectum Gangliosides and Glycoproteins of Chickens in Darkness or Exposed to Light ...
We have recently shown that in addition to the previously described binding partner ganglioside GM1, CTB binds to fucosylated ...
Repeated injection of gangliosides alone in human or in mouse is not enough for inducing the synthesis of anti-ganglioside ... Figure 2: Schematic representation of the main pathway of O-acetyl-GD2 ganglioside biosynthesis. GD2 ganglioside is synthesized ... 3. Metabolism of O-Acetyl-GD2 Ganglioside. The modification of ganglioside expression in oncogenesis is mainly associated with ... a specific ganglioside profile, which is modified during embryogenesis, and ontogenesis . Some ganglioside species further ...
If you would like to schedule an appointment with one of our nationally ranked specialists or Primary Care physicians please click or call 800-881-7385.. ...
Pathogenicity of anti-ganglioside antibodies in the Guillain-Barré syndrome.. van Sorge NM1, van der Pol WL, Jansen MD, van den ... Antibodies directed against glycolipid structures (gangliosides), which are highly expressed in the peripheral nervous system, ...
GDAP2 ganglioside induced differentiation associated protein 2 [Homo sapiens] GDAP2 ganglioside induced differentiation ... ganglioside induced differentiation associated protein 2provided by HGNC. Primary source. HGNC:HGNC:18010 See related. Ensembl: ... GDAP2 ganglioside induced differentiation associated protein 2 [ Homo sapiens (human) ] Gene ID: 54834, updated on 12-Oct-2019 ... NM_001135589.3 → NP_001129061.1 ganglioside-induced differentiation-associated protein 2 isoform b ...
Here, we report that ganglioside GD2 (a glycosphingolipid) identifies a small fraction of cells in human breast cancer cell ...
Ganglioside GM1 (GM1) micelles have been studied by means of water proton T1 NMRD experiment. The ﬁeld dependent spin-lattice ... A Proton Water T1-NMRD Study of Ganglioside Micelles. Westlund, Per-Olof Umeå University, Faculty of Science and Technology, ... Proton T1.NMRD Profiels, Proton Spin-lattice Relaxation, Ganglioside Micelles National Category Chemical Sciences Identifiers. ...
References for Abcams Anti-Ganglioside GD3 antibody [4F6] (ab6217). Please let us know if you have used this product in your ...
... the peripheral nerve gangliosides, authentic GM1, bovine brain ganglioside mixture (Cronassial; Fidia, Padova, Italy), and each ... d) Anti-ganglioside Ab from rabbits that developed limb weakness after sensitization with C. jejuni LOS. Of the bovine brain ... a) Carbohydrate mimicry of GM1 ganglioside by the LOS of C. jejuni (CF 90-26) from a GBS patient. GM1 is located in the nerve ... The anti-GM1 mAb bound to the GM1 of human peripheral nerve gangliosides on a TLC plate. A point worth noting is that GM1 is ...
AntibodiesAccumulationMiceBrainAbstractGD1aExpression of gangliosideSerumGT1bReceptorsRole of gangliosidesMetabolismGlycosphingolipidTotal gangliosideTissuesImmunologyAlterations in ganglioside expressionLipidsPlasma membraneMoleculeNeuronsGlycolipidGD1bRefers to gangliosidePathologyTumorNeuronalExogenousAnti-Human GD3Sialic acid residuesGroups of gangliosidesImportance of gangliosidesBiosynthesis of gangliosidesActivatorAffinityMelanoma cellsPathogenesisNeurologicalInhibitMurineNovel gangliosideDifferent gangliosidesIndividual gangliosidesCholera toxinCompositionGlycosphingolipids that containSpeciesGlobosidesResiduesMass spectrometryProteinsHydrolysisCarbohydrateNeuAcParkinson's DiseaseBrain and spinal cord
- N-butyldeoxygalactonojirimycin reduces brain ganglioside and GM2 content in neonatal Sandhoff disease mice. (ox.ac.uk)
- NB-DGJ treatment, administered from postnatal day 2 (p-2) to p-5 (600 mg/kg/day)), significantly reduced total brain ganglioside and GM2 content in the Sandhoff disease (Hexb(-/-)) mice, but did not reduce the content of GA2. (ox.ac.uk)
- mice abnormally accumulate GM2 and GA2 ganglioside and serve as a model of Sandhoff disease. (nih.gov)
- abstract = "Cellular location of ganglioside-sialidase activity was determined in confluent hamster embryo fibroblasts transformed with herpes simplex virus type 2. (elsevier.com)
- abstract = "Various reports have appeared indicating that the expression of gangliosides may be largely regulated at the genetic level. (elsevier.com)
- The major groups of gangliosides found in mammalian brains are GM1, GD1a, GT1b and GD1b. (humpath.com)
- Thus glycans of gangliosides such as GD1a and GM1 are functional counterreceptors by a pairing with tissue lectins, an emerging aspect of ganglioside and lectin functionality. (uni-muenchen.de)
- The gangliosides GD1a, GM1, and asialo-GM1 (GA1) are natural components of murine macrophages. (asm.org)
- MNV-1 bound to ganglioside GD1a, which is characterized by an SA on the terminal galactose, but not to GM1 or asialo-GM1 in an enzyme-linked immunosorbent assay. (asm.org)
- We determined the ability of mixed gangliosides (16% GD1b, 19% GT1b, 21% GM1, and 40% GD1a) and individual gangliosides GM1 and GD1b to modulate the NV-PLA 2 induced human erythrocyte ghost membrane damage. (academicjournals.org)
- Paired sample analysis of ascitic fluid and plasma from the same patients confirmed the significant expression of anti-GD1a IgM in OEC patients, while no such difference was observed with other anti-ganglioside IgMs among different groups. (curehunter.com)
- The significance of the endogenous IgM response to GD1a may be to eliminate this immunosuppressive-ganglioside from the tumor-microenvironment. (curehunter.com)
- It acts at least on GD1a, GM1, GM2 and GM3 to catalyzes the reaction of hydrolysis of ganglioside to lysoganglioside and a fatty acid. (justia.com)
- This enzyme has been decided to be a novel enzyme having physico-chemical properties and has a novel enzyme action in that it does not utilize Gal-cer, Glc-cer Lac-cer, Gb3-cer and asialoGM1 as substrate, but utilizes at least GD1a, GM1, GM2 and GM3 as substrate and hydrolyzes ceramide in the molecule of this ganglioside into sphingosine base and fatty acid to produce lysoganglioside and fatty acid. (justia.com)
- Somogyi A, Petcherski A, Beckert B, Huebecker M, Priestman DA, Banning A, Cotman SL, Platt FM, Ruonala MO, Tikkanen R. Altered Expression of Ganglioside Metabolizing Enzymes Results in GM3 Ganglioside Accumulation in Cerebellar Cells of a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis. (harvard.edu)
- Altered expression of ganglioside GM3 molecular species and a potential regulatory role during myoblast differentiation. (harvard.edu)
- Here, we aim to investigate the local expression of ganglioside GD2, as the recently proposed breast CSC (BCSC) marker, through the assessment of GD2 synthase mRNA, the key enzyme involved in GD2 synthesis. (omicsonline.org)
- 8762-8769, 1999), we established a subclone of the rat dorsal root ganglion-derived F-11 cells in which the expression of ganglioside GD3 was inhibited by stable transfection of the antisense vector against CMP-NeuAc:GM3 α2-8 sialyltransferase (GD3-synthase) gene. (aacrjournals.org)
- Shedding of the FucG M1 ganglioside was studied both in vitro and in vivo using SCLC cell lines and nude mice xenografts of SCLC cells as model systems, and finally immunochemical analyses were performed on serum samples from patients with SCLC. (aacrjournals.org)
- Background Serum IgM binding to GM1 ganglioside (GM1) is often associated with chronic acquired motor neuropathies. (bmj.com)
- The results suggest that gangliosides, particularly GT1b, may play a role in the pathologic mechanisms of TAO by stimulating an increase in hyaluronic acid. (arvojournals.org)
- As an alternate technique, DPPC/GT1b or total ganglioside extract (80:20) monolayers of a Langmuir Blodgett (LB) Trough were adapted to be artificial membrane models for toxin insertion studies. (byu.edu)
- In the case of the insulin receptor, sialylparagloboside and the structurally related GM3 ganglioside have been found to inhibit the intrinsic tyrosine kinase activity of soluble receptors ( 6 , 7 ). (pnas.org)
- For example, the monosialylated GM1 ganglioside is a critical organizer of lipid rafts, which act as hubs for receptors and signaling complexes. (jneurosci.org)
- At the cellular level, gangliosides influence cell growth and death, probably because they are involved in the lipid-mediated assembly of signaling molecules such as growth factor receptors or integrins on the membranes. (eurekaselect.com)
- Gangliosides perform important functions through carbohydrate-specific interactions with proteins, for example, as receptors in cell-cell recognition, which can be exploited by viruses and other pathogens, and also by regulating signaling proteins, such as the epidermal growth factor receptor (EGFR) and the vascular endothelial growth factor receptor (VEGFR), through lateral interaction in the membrane. (frontiersin.org)
- In conclusion, our data indicate that MNV can use terminal SA on gangliosides as attachment receptors during binding to murine macrophages. (asm.org)
- We show that decreasing Abl family kinase activity results in low levels of cell surface ganglioside receptors for mouse polyomavirus and that inhibition of sialidase activity promotes virion binding in the absence of Abl family kinase activity. (elsevier.com)
- In contrast to LT-IIb, which binds strongly to ganglioside receptors decorated with either N -acetylneuraminic acid (NeuAc) or N -glycolylneuraminic acid (NeuGc), LT-IIb(T13I) binds NeuAc gangliosides much less well. (pubmedcentralcanada.ca)
- To determine if the adjuvant properties of LT-IIb(T13I) are altered in the absence of NeuGc ganglioside receptors, experiments were conducted using a Cmah -null mouse line which is deficient in the synthesis of NeuGc gangliosides. (pubmedcentralcanada.ca)
- The aim of this study was to determine the role of gangliosides in pathogenic mechanisms of thyroid-associated ophthalmopathy (TAO). (arvojournals.org)
- In this thesis, the role of gangliosides in glial proliferation, migration and differentiation as well as the regeneration of the olfactory system and myelination were studied using mice lacking enzymes involved in ganglioside biosynthesis. (gla.ac.uk)
- The potential physiological role(s) of gangliosides on the regulation of extracellular nucleotide metabolism are discussed. (eurekaselect.com)
- Miki Hara-Yokoyama, " Gangliosides Potentially Inhibit Extracellular Nucleotide Metabolism", Current Medicinal Chemistry (2006) 13: 2233. (eurekaselect.com)
- Although the metabolism of the lipid class "ganglioside" is linked to AD their role in oligomerization is controversial. (cas.cz)
- Yu, RK 1994, ' Development regulation of ganglioside metabolism ', Progress in Brain Research , vol. 101, no. (elsevier.com)
- These mutant mice were unable to synthesize GM3 ganglioside, a simple and widely distributed glycosphingolipid. (pnas.org)
- Here, we report that ganglioside GD2 (a glycosphingolipid) identifies a small fraction of cells in human breast cancer cell lines and patient samples that are capable of forming mammospheres and initiating tumors with as few as 10 GD2+ cells. (jci.org)
- The functions of gangliosides as specific determinants suggest its important role in the growth and differentiation of tissues as well as in carcinogenesis. (wikipedia.org)
- It is the most widely distributed ganglioside among tissues, and it serves as a precursor for most of the more complex ganglioside species. (pnas.org)
- One example, the GM3 ganglioside, abundant in almost all healthy tissues, is shown in Figure 1 . (frontiersin.org)
- In the adult Xenopus, XI characterizes the ganglioside pattern of the central nervous system while is absent in all the other tested tissues. (biomedsearch.com)
- Gangliosides can amount to 6% of the weight of lipids from brain, but they are found at low levels in all animal tissues. (hmdb.ca)
- The gangliosides profile and mRNA level of sialyltransferases of the orbital tissues from TAO patients ( n = 5) and non-TAO subjects ( n = 4) were investigated. (arvojournals.org)
- Gangliosides are found in cell surface membranes of mammalian tissues and organs and are especially rich in the neural tissues. (justia.com)
- The name ganglioside was first applied by the German scientist Ernst Klenk in 1942 to lipids newly isolated from ganglion cells of the brain. (wikipedia.org)
- Gangliosides are a family of membrane lipids enriched in the central nervous system (CNS) that play an important role in cell signaling processes on the membrane. (uwo.ca)
- However, heavily glycosylated, low abundance and chemically unusual lipids such as the gangliosides tend to be neglected in otherwise thorough lipidomic studies. (gla.ac.uk)
- Recently, using the sensitivity of single molecule fluorescence and taking advantage of model membrane systems containing essential lipids with well-defined compositions, we showed that physiological levels of ganglioside GM1 prevent in-membrane oligomerization of Ab. (cas.cz)
- Gangliosides, sialic acid containing glycosphingolipids, are normal components of the plasma membrane of vertebrate cells and are particularly abundant in the nervous system. (springer.com)
- Our results suggest that the ganglioside, GRIFIN, and alphaA-crystallin exist as a complex within the fiber cell plasma membrane. (arvojournals.org)
- Different regions of the brain have differing levels of vulnerability to stress and injury, and this brain region-dependent variability to vulnerability could be partly explained by the existence of glycosphingolipids within the cell's plasma membrane called gangliosides. (uwo.ca)
- These data provide evidence that Abl family kinases reduce ganglioside turnover in the plasma membrane by inhibiting host cell sialidase activity. (elsevier.com)
- Approximately equal specific activities of ganglioside-sialidase activity were found to be associated with the crude lysosomal and crude plasma membrane fractions isolated from whole cell homogenates. (elsevier.com)
- Whole transformed cells hydrolyzed exogenous ganglioside substrate, suggesting a partial location of the cellular sialidase on the outer surface of the plasma membrane of these cells. (elsevier.com)
- These results show that, in herpes simplex virus- transformed fibroblasts, ganglioside-sialidase is both a lysosomal and a plasma membrane enzyme. (elsevier.com)
- The letter G refers to ganglioside, and the subscripts M, D, T and Q indicate that the molecule contains mono-, di-, tri and quatra-sialic acid. (hmdb.ca)
- A procedure is described for the preparation of Tay-Sachs ganglioside specifically labeled in the sialic acid portion of the molecule. (eurekamag.com)
- Radioactive gangliosides were isolated and selectively degraded with bacterial neuraminidase and rat liver beta-galactosidase to Tay-Sachs ganglioside-(3)H. Radioactivity in the labeled product was confined to the N-acetyl-neuraminic acid portion of the molecule. (eurekamag.com)
- GM2 ganglioside accumulates in the lysosomal storage disease, so-called Tay-Sachs disease and is degraded in the lysosome by a series of enzymes, beginning at the non-reducing terminal of the molecule. (justia.com)
- Cholera Tetanus Botulism Leprosy Obesity, where inadequate ganglioside expression in mediobasal hypothalamic neurons deregulates neuronal leptin and insulin signaling. (wikipedia.org)
- To investigate whether injury-induced ganglioside processing is operative in CNS neurons, cultured retinal neurons were axotomized and analyzed for GM1 content. (jneurosci.org)
- They were discovered by Ernst Klenk in the 1940s, who proposed the term "ganglioside" due to the abundance of these molecules in "Ganglionzellen" (neurons). (frontiersin.org)
- One of such antigens is GD2 ganglioside which is abundantly expressed on the surface of neuroectodermally-derived tumor cells, whereas on normal cells its presence is restricted to neurons, peripheral pain fibers, and skin melanocytes. (buffalo.edu)
- Factors involved in the regulation of ganglioside expression include the proper translocation and sorting of the glycolipid products in multi-glycosyltransferase systems. (elsevier.com)
- Acta 176, 339-347 (1969), Science 178, 1100-1102 (1972)], but this ceramidase does not hydrolyze the linkage between a sphingosine base and the fatty acid of a glycolipid like ganglioside. (justia.com)
- Mixed gangliosides (18 nM) and GM1 (15 nM) offered 81 and 86% protection respectively, whereas GD1b (20 nM) did not show significant protection. (academicjournals.org)
- Mixed gangliosides restored the Na + K + ATPase activity by 78%, whereas GM1 and GD1b offered 74 and 52% restoration respectively. (academicjournals.org)
- Mixed gangliosides and GM1 exhibited a significant dose dependent inhibition of NV-PLA 2 activity when compared to GD1b. (academicjournals.org)
- The data show that mixed gangliosides and GM1 were effective in modulating NV-PLA 2 induced erythrocyte membrane damage than GD1b. (academicjournals.org)
- Detection and characterization of N-glycolyated gangliosides in Wilms tumor by immunohistochemistry," Pediatric and Developmental Pathology , vol. 13, no. 1, pp. 18-23, 2010. (hindawi.com)
- Ganglioside dysregulation has been implicated as an important mechanisms driving pathology in the aging brain, yet there is little information on where and when these lipid changes occur as well as the role of dysregulation during neurodegeneration. (uwo.ca)
- CQ was found to prevent ganglioside dysregulation acutely after stroke and was correlated with reduced pathology and functional impairments. (uwo.ca)
- The results demonstrate that complex ganglioside storage, while responsible for much of the neuronal pathology, does not significantly influence the clinical phenotype of the NP-C model. (oup.com)
- It has been found that tumor formation can induce the synthesis of a new complement of ganglioside, and very low concentrations of a specific ganglioside can induce differentiation of cultured neuronal tumor cells. (wikipedia.org)
- Through both mechanisms, tumor-associated gangliosides may affect malignant progression, which makes them attractive targets for cancer immunotherapies. (frontiersin.org)
- Ganglioside GD3 is overexpressed in many types of tumors and may be associated with tumor progression and the development of metastatic potential. (aacrjournals.org)
- The tumor-associated ganglioside GD2 is an attractive target for immunotherapy. (biomedcentral.com)
- However, gangliosides are weakly immunogenic and are considered inadequate for generating tumor-specific cellular responses. (buffalo.edu)
- This approach led to isolation of non-overlapping peptides that exhibited mimicry with GD2 ganglioside, based on their abilities to inhibit the binding of GD2-specific mAbs to GD2-positive tumor cells. (buffalo.edu)
- In conclusion, the presented data suggest that (i) inter-conversion of weakly immunogenic GD2 ganglioside into peptide mimics leads to the breaking of tolerance in the immunized animals and therefore might be useful in active, specific immunotherapy against GD2-positive tumors, and (ii) multiple apoptotic pathways can be triggered by GD2-specific mAbs in GD2 + tumor cells. (buffalo.edu)
- Although the most accepted theory indicates that PaBV directly triggers an inflammatory response in this disease, another hypothesis suggests the disease is triggered by autoantibodies targeting neuronal gangliosides, and PDD might therefore resemble Guillain-Barré Syndrome (GBS) in its pathogenesis. (peerj.com)
- This article addresses the structural similarity between the tandem sialic acid residues of gangliosides and nicotinamide adenine dinucleotide (NAD+) determined from biochemical data showing that gangliosides inhibit NAD+ glycohydrolase activity and theoretical considerations. (eurekaselect.com)
- Gangliosides were later classified by Svennerholm according to the number of sialic-acid residues and chromatographic mobility ( 1 ). (frontiersin.org)
- NeuNAc, an acetylated derivative of the carbohydrate sialic acid, makes the head groups of gangliosides anionic at pH 7, which distinguishes them from globosides. (wikipedia.org)
- NeuNAc, an acetylated derivative of the carbohydrate sialic acid, makes the head groups of Gangliosides anionic. (hmdb.ca)
- G(M2) Ganglioside accumulates due to a deficiency of hexosaminidase A or B (beta-N-acetylhexosaminidase), or GM2 activator protein, resulting in Gangliosidoses. (hmdb.ca)
- Recombinant human Ganglioside GM2 activator protein Human samples 80 % of the research is conducted on human samples. (antibody-antibodies.com)
- However, it has been reported that a specific activator is required for catabolism of GM2 ganglioside with .beta. (justia.com)
- Enzymatic interconversion of ganglioside glycans by sialidase action, relevant for neuroblastoma cell differentiation and growth control in vitro, for axonogenesis and axon regeneration, as well as for proper communication between effector and regulatory T cells, changes lectin-binding affinity profoundly. (uni-muenchen.de)
- Orchestrations of loss/gain of affinity, of ganglioside/lectin expression, and of lectin presence in a network offer ample opportunities for fine-tuning. (uni-muenchen.de)
- Botulinum neurotoxin serotype A insertions into the LB monolayers in the presence of BoNT/A low affinity ganglioside receptor alone, independent of pH. (byu.edu)
- Thomas CP, Buronfosse A, Combaret V, Pedron S, Fertil B, Portoukalian J. Gangliosides protect human melanoma cells from ionizing radiation-induced clonogenic cell death. (acris-antibodies.com)
- Zebda N, Pedron S, Rebbaa A, Portoukalian J, Berthier-Vergnes O. Deficiency of ganglioside biosynthesis in metastatic human melanoma cells: relevance of CMP-NeuAc:LacCer alpha 2-3 sialyltransferase (GM3 synthase). (acris-antibodies.com)
- Does not cross-react with other gangliosides purified from neuroblastoma or melanoma cells. (lsbio.com)
- These results and the finding of enhanced expression of GM3 synthase in models of insulin resistance have led to the suggestion that GM3 ganglioside overexpression may play a role in the pathogenesis of type 2 diabetes through a negative modulation on insulin receptor signaling ( 6 ). (pnas.org)
- Carbohydrate mimicry [Galβ1-3GalNAcβ1-4(NeuAcα2-3)Galβ1-] between the bacterial lipooligosaccharide and human GM1 ganglioside is seen as having relevance to the pathogenesis of Guillain-Barré syndrome, and conclusive evidence is reported here. (pnas.org)
- Cell-mediated immunity (CMI) to myelin components has been implicated in Multiple Sclerosis (MS) pathogenesis: two targets were suggested, Myelin Basic Protein with controversial results and, more recently, gangliosides. (semanticscholar.org)
- Animals without gangliosides display numerous neurological impairments (motor function loss, depression-lie behaviorl, congnitive impairment) and the University of Alberta researchers hypothesized that the addition of GM1 may improve some of the symptoms on display in HD. (raredr.com)
- Zika virus infection is associated with the development of Guillain-Barré syndrome (GBS), a neurological autoimmune disorder caused by immune recognition of gangliosides and other components at nerve membranes. (escholarship.org)
- This invention relates to a novel ganglioside ceramidase and a process for producing it. (justia.com)
- Recent data demonstrated that sera from some patients with GBS react with ganglioside complexes (GSCs) consisting of two different gangliosides, but not with each constituent ganglioside. (unboundmedicine.com)
- Gangliosides along with other components as cholesterol are known to form lipid rafts, in which two different gangliosides may form a new conformational epitope. (unboundmedicine.com)
- The carbohydrate portion of the ganglioside GM1 is the site of attachment of cholera toxin, the protein secreted by Vibrio cholerae. (hmdb.ca)
- Cholera toxin B (CTB) subunit has been taken as a model system and its binding with several gangliosides has been demonstrated using this sensor. (tamu.edu)
- The ability of Fuc-GM1 ganglioside to mimic the receptor function of GM1 for cholera toxin (CT) has been investigated. (elsevier.com)
- Gazzotti G, Sonnino S, Ghidoni R, Orlando P, Tettamanti G (1984b) Preparation of the tritiated molecular forms of gangliosides, separated with homogeneous long chain base composition. (springer.com)
- The elucidation of the chemical composition of sialic acid was accompanied by the recognition that it has specific biological properties and that the gangliosides are frequently the glycoconjugate carrier. (springer.com)
- Almost 200 gangliosides species have been described, showing differences in the number, the order, and the linkage of the glycosyl and sialyl residues [ 4 ]. (hindawi.com)
- Phylogenetic analysis of brain gangliosides using the A2B5 preparation demonstrated that c-series gangliosides are enriched in lower animals, especially bony fish of different species. (nih.gov)
- Understanding the precise nature of native gangliosides, including low abundance species and modifications, is an important first step. (gla.ac.uk)
- Matrix-Assisted Laser Desorption/Ionization (MALDI) imaging mass spectrometry (IMS) is a novel imaging technique that can map the distribution of ionizable molecules on a sample in a 2-dimensional format, making it the ideal tool for analyzing gangliosides on post-mortem brain tissue sections. (uwo.ca)
- Caughlin, Sarah, "Examining the role of ganglioside homeostasis in neurodegeneration and aging using MALDI imaging mass spectrometry" (2018). (uwo.ca)
- Liquid chromatography electrospray ionization mass spectrometry LC-ESI-MS was used to quantify ganglioside levels following activation, and immunofluorescence was used to stain for GM1, GM2 and GM3 gangliosides. (uwo.ca)
- It was the aim here to optimise separation and mass spectrometry methodologies for ganglioside analysis. (gla.ac.uk)
- Finally the recently developed technique of imaging mass spectrometry, using matrix assisted laser desorption ionisation (MALDI) and secondary ion mass spectrometry (SIMS) ion sources, was investigated for its capability for direct ganglioside analysis in brain and spinal cord tissue sections. (gla.ac.uk)
- We have recently shown that in addition to the previously described binding partner ganglioside GM1, CTB binds to fucosylated proteins. (gu.se)
- Ganglioside-embedding small bicelles for probing membrane-landing processes of intrinsically disordered proteins. (sigmaaldrich.com)
- We successfully displayed a series of gangliosides on small bicelles with a uniform confined size, offering nanoscale standardized membrane mimics for spectroscopic characterization of weak encounter complexes formed between ganglioside clusters and amyloidogenic proteins. (sigmaaldrich.com)
- In this review, we describe how proteins recognize gangliosides, focusing on the molecular recognition of gangliosides associated with cancer immunotherapy, and discuss the importance of these molecules in cancer research. (frontiersin.org)
- Their spatial accessibility and the dynamic regulation of their profile are factors that argue in favor of a role of glycans of gangliosides as ligands (counterreceptors) for carbohydrate-binding proteins (lectins). (uni-muenchen.de)
- Immunoprecipitation of the ganglioside from the 25/45 fraction resulted in the co-precipitation of both galectin related inter-fiber protein (GRIFIN) and alphaA-crystallin, two important structural proteins within the ocular lens. (arvojournals.org)
- The inventors have succeeded in the isolation and purification of an enzyme which produces lysoganglioside GM2 and a fatty acid by hydrolysis of GM2 ganglioside as a substrate from culture of microorganism N285 strain belonging to genus Nocardia collected and isolated from forest soil in Himeji-shi in Hyogo-ken, Japan. (justia.com)
- The ganglioside G-4 was shown to have the following structure: NeuAc-NeuAc-NeuAc-Galbeta1-3Gal NAcbeta1-4(NeuAc-NeuAc-NeuAcalpha2-3)Galbeta1-4Glcbeta1-1'Cer. (nih.gov)
- Reacts with GD3 Ganglioside: (NeuAc alpha 2-8NeuAc alpha 2-3Gal beta 1-4Glc beta 1-1Cer). (acris-antibodies.com)
- Cross reacts with 9-O-AcGD3 ganglioside (9OAcNeuAc alpha 2-8NeuAc alpha 2-3Gal beta 1-4Glc beta 1-1Cer) and GT1a ganglioside (NeuAc alpha 2-8NeuAc alpha 2-3Gal beta 1-4GalNAc beta 1-4 (NeuAc alpha 2-3) Gal beta 1-4Glc beta 1-1Cer). (acris-antibodies.com)
- Recently, the ganglioside FucG M1 (Fucα1-2Galβ1-3GalNAcβ1-4[NeuAcα2-Galβ1-4Glcβ1-1Cer) was identified as a small cell lung cancer (SCLC) marker both in chemical and histochemical studies. (aacrjournals.org)