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 with a sulfate group esterified to one of the sugar groups.
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.
An enzyme that catalyzes the hydrolysis of cerebroside 3-sulfate (sulfatide) to yield a cerebroside and inorganic sulfate. A marked deficiency of arylsulfatase A, which is considered the heat-labile component of cerebroside sulfatase, has been demonstrated in all forms of metachromatic leukodystrophy (LEUKODYSTROPHY, METACHROMATIC). EC 3.1.6.8.
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)
An autosomal recessive metabolic disease caused by a deficiency of CEREBROSIDE-SULFATASE leading to intralysosomal accumulation of cerebroside sulfate (SULFOGLYCOSPHINGOLIPIDS) in the nervous system and other organs. Pathological features include diffuse demyelination, and metachromatically-staining granules in many cell types such as the GLIAL CELLS. There are several allelic and nonallelic forms with a variety of neurological symptoms.
A class of Echinodermata characterized by long, slender bodies.
A paleotropical genus of fungi in the family Tricholomataceae. They are obligate symbionts of termites.
Cerebrosides which contain as their polar head group a galactose moiety bound in glycosidic linkage to the hydroxyl group of ceramide. Their accumulation in tissue, due to a defect in beta-galactosidase, is the cause of galactosylceramide lipidosis or globoid cell leukodystrophy.
An enzyme that catalyzes the conversion of UDP-galactose and N-acylsphingosine to D-galactosylceramide and UDP.
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.
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 genus of fleshy shelf basidiomycetous fungi, family Schizophyllaceae, order POLYPORALES, growing on woody substrata. It is pathogenic in humans.
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.
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)
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.
A sebaceous gland that, in some animals, acts as an accessory to the lacrimal gland. The harderian gland excretes fluid that facilitates movement of the third eyelid.
An autosomal recessive disorder caused by a deficiency of acid beta-glucosidase (GLUCOSYLCERAMIDASE) leading to intralysosomal accumulation of glycosylceramide mainly in cells of the MONONUCLEAR PHAGOCYTE SYSTEM. The characteristic Gaucher cells, glycosphingolipid-filled HISTIOCYTES, displace normal cells in BONE MARROW and visceral organs causing skeletal deterioration, hepatosplenomegaly, and organ dysfunction. There are several subtypes based on the presence and severity of neurological involvement.
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)
The lipid-rich sheath surrounding AXONS in both the CENTRAL NERVOUS SYSTEMS and PERIPHERAL NERVOUS SYSTEM. The myelin sheath is an electrical insulator and allows faster and more energetically efficient conduction of impulses. The sheath is formed by the cell membranes of glial cells (SCHWANN CELLS in the peripheral and OLIGODENDROGLIA in the central nervous system). Deterioration of the sheath in DEMYELINATING DISEASES is a serious clinical problem.
A group of four homologous sphingolipid activator proteins that are formed from proteolytic cleavage of a common protein precursor molecule referred to as prosaposin.
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 genus of STARFISH in the family Asteriidae. One species, Asterias rubens, is the most common in the north-east Atlantic region.
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.
Compounds possessing both a hydroxyl (-OH) and an amino group (-NH2).
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)
Organic compounds containing both the hydroxyl and carboxyl radicals.
Loss of water by diffusion through the skin and by evaporation from the respiratory tract.
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.
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.
Enzymes which transfer sulfate groups to various acceptor molecules. They are involved in posttranslational sulfation of proteins and sulfate conjugation of exogenous chemicals and bile acids. EC 2.8.2.
Inorganic and organic derivatives of sulfuric acid (H2SO4). The salts and esters of sulfuric acid are known as SULFATES and SULFURIC ACID ESTERS respectively.
A plant genus of the family STERCULIACEAE. S. urens is the source of KARAYA GUM which is sometimes called Indian tragacanth, which is different from the true TRAGACANTH which comes from ASTRAGALUS GUMMIFER.
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.
An arylsulfatase with high specificity towards sulfated steroids. Defects in this enzyme are the cause of ICHTHYOSIS, X-LINKED.
Differentiated tissue of the central nervous system composed of NERVE CELLS, fibers, DENDRITES, and specialized supporting cells.
Enzymes that catalyze the hydrolysis of a phenol sulfate to yield a phenol and sulfate. Arylsulfatase A, B, and C have been separated. A deficiency of arylsulfatases is one of the causes of metachromatic leukodystrophy (LEUKODYSTROPHY, METACHROMATIC). EC 3.1.6.1.
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.
A key enzyme in SPHINGOLIPIDS biosynthesis, this enzyme catalyzes the pyridoxal-5'-phosphate-dependent condensation of L-SERINE and PALMITOYL COENZYME A to 3-dehydro-D-sphinganine. The enzyme consists of two different subunits.
Works containing information articles on subjects in every field of knowledge, usually arranged in alphabetical order, or a similar work limited to a special field or subject. (From The ALA Glossary of Library and Information Science, 1983)
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)
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.
Derivatives of phosphatidic acids in which the phosphoric acid is bound in ester linkage to a choline moiety. Complete hydrolysis yields 1 mole of glycerol, phosphoric acid and choline and 2 moles of fatty acids.
The principal sterol of all higher animals, distributed in body tissues, especially the brain and spinal cord, and in animal fats and oils.
A fluorescent compound that emits light only in specific configurations in certain lipid media. It is used as a tool in the study of membrane lipids.
Layers of lipid molecules which are two molecules thick. Bilayer systems are frequently studied as models of biological membranes.
Lipids containing one or more phosphate groups, particularly those derived from either glycerol (phosphoglycerides see GLYCEROPHOSPHOLIPIDS) or sphingosine (SPHINGOLIPIDS). They are polar lipids that are of great importance for the structure and function of cell membranes and are the most abundant of membrane lipids, although not stored in large amounts in the system.
The interchange of goods or commodities, especially on a large scale, between different countries or between populations within the same country. It includes trade (the buying, selling, or exchanging of commodities, whether wholesale or retail) and business (the purchase and sale of goods to make a profit). (From Random House Unabridged Dictionary, 2d ed, p411, p2005 & p283)
Governmental levies on property, inheritance, gifts, etc.
The charge levied on the consumer for drugs or therapy prescribed under written order of a physician or other health professional.
Study of stamps or postal markings. It usually refers to the design and commemorative aspects of the stamp.
Traditional Arabic methods used in medicine in the ARAB WORLD.

Partial purification and properties of porcine thymus lactosylceramide beta-galactosidase. (1/288)

Porcine thymus lactosylceramide beta-galactosidase was purified by a simple procedure. In the final step of isoelectric focusing the enzyme was separated into two peaks of pI 6.3 (peak I) and 7.0 (peak II), which showed 3,600- and 4,000-fold enhancement of lactosylceramide-hydrolysing activity, respectively. The two peaks had identical mobility on polyacrylamide gel electrophoresis. The apparent molecular weight was 34,000. Neither monosialoganglioside (GM1) nor galactosylceramide was hydrolysed by the purified enzyme fractions. The optimal pH was at 4.6, and sodium taurocholate was essential for the reaction. The apparent Km was 2.3 x 10-5 M. The reaction was stimulated by sodium chloride and linoleic acid, while it was strongly inhibited by Triton X-100 and bovine serum albumin. Galactosylceramide, p-nitrophenyl beta-galactoside, and p-nitrophenol were weak inhibitors. No effects of GM1 and galactose were observed on the hydrolysis of lactosylceramide.  (+info)

Synthesis and turnover of cerebrosides and phosphatidylserine of myelin and microsomal fractions of adult and developing rat brain. (2/288)

The synthesis and turnover of cerebrosides and phospholipids was followed in microsomal and myelin fractions of developing and adult rat brains after an intracerebral injection of [U-14C]serine. The kinetics of incorporation of radioactivity into microsomal and myelin cerebrosides indicate the possibility of a precursor-product relationship between cerebrosides of these membranes. The specific radioactivity of myelin cerebrosides was corrected for the deposition of newly formed cerebrosides in myelin. Multiphasic curves were obtained for the decline in specific radioactivity of myelin and microsomal cerebrosides, suggesting different cerebroside pools in these membranes. The half-life of the fast turning-over pool of cerebrosides of myelin was 7 and 22 days for the developing and adult rat brain respectively. The half-life of the slowly turning-over pool of myelin cerebrosides was about 145 days for both groups of animals. The half-life of the rapidly turning-over microsomal cerebrosides was calculated to be 20 and 40 h for the developing and adult animals respectively. The half-life of the intermediate and slowly turning-over microsomal cerebrosides was 11 and 60 days respectively, for both groups of animals. The amount of incorporation of radioactivity into microsomal cerebrosides from L-serine was greatly decreased in the adult animals, and greater amounts of the precursor were directed towards the synthesis of phosphatidylserine. In the developing animals, considerable amounts of cerebrosides were synthesized from L-serine, besides phosphatidylserine. The time-course of incorporation indicated that a precursor-product relationship exists between microsomal and myelin phosphatidylserine. The half-life of microsomal phosphatidylserine was calculated to be about 8 h for the fast turning-over pool in both groups of animals.  (+info)

Divalent cation-mediated interaction between cerebroside sulfate and cerebrosides: an investigation of the effect of structural variations of lipids by electrospray ionization mass spectrometry. (3/288)

Divalent cations mediate a carbohydrate-carbohydrate association between the two major glycolipids, galactosylceramide (GalCer) and its sulfated form, cerebroside sulfate (CBS), of the myelin sheath. We have suggested that interaction between these glycolipids on apposed extracellular surfaces of myelin may be involved in the stability or function of this multilayered structure. A mutant mouse lacking galactolipids because of a disruption in the gene that encodes a galactosyltransferase forms myelin that initially appears relatively normal but is unstable. This myelin contains glucosylceramide (GlcCer) instead of GalCer. To better understand the role of GlcCer in myelin in this mutant, we have compared the ability of divalent cations to complex CBS (galactosyl form) with GlcCer or GalCer in methanol solution by using positive ion electrospray ionization mass spectrometry. Because both the alpha-hydroxylated fatty acid species (HFA) and the nonhydroxylated fatty acid species (NFA) of these lipids occur in myelin, we have also compared the HFA and NFA species. In addition to monomeric Ca2+ complexes of all three lipids and oligomeric Ca2+ complexes of both GalCer and GlcCer, Ca2+ also caused heterotypic complexation of CBS to both GalCer and GlcCer. The heterotypic complexes had the greatest stability of all oligomers formed and survived better at high declustering potentials. Complexes of CBS with GlcCer were less stable than those with GalCer. This was confirmed by using the free sugars and glycosides making up the carbohydrate headgroups of these lipids. HFA species of CBS and GalCer formed more stable complexes than NFA species, but hydroxylation of the fatty acid of GlcCer had no effect. The ability of GlcCer to also complex with CBS, albeit with lower stability, may allow GlcCer to partially compensate for the absence of GalCer in the mouse mutant.  (+info)

Forssman penta- and tetraglycosylceramide are xenoantigens of ostrich kidney and liver. (4/288)

The heterophile antigens Galalpha1-->3Gal and N-glycolylneuraminic acid are the major obstacle to grafting mammal organs, especially from pig, to man. Lack of expression of these common xenoantigens by birds has raised interest in ostrich as a potential organ donor for xenotransplantation. Glycosphingolipids of ostrich liver and kidney were investigated for their carbohydrate determinants. Both organs were found similar in their glycolipid composition with three major species, mono-, di-, and pentaglycosylceramide. The pentaglycosylceramide was characterized as the Forssman antigen. In both organs, the ceramide portion was highly hydroxylated with prevalence of alpha-hydroxylated fatty acids, C18 phytosphingosine in kidney and C18 sphingosine in liver Forssman glycolipid. These data indicate that hydroxylation of kidney glycosphingolipids, which is found in mammals, has been maintained since the divergence of birds from other vertebrates. Characterization of a minor glycolipid as a Forssman tetraglycosylceramide built on the galabiosylceramide core indicates that the Forssman tetraglycosylceramide also exists in vivo. Its precursors, galactosyl- and galabiosylceramide, were characterized in kidney and liver. The Forssman antigen is the third heterophile antigen against which man raises natural antibodies. Its localization in the vascular endothelium and connective tissue makes ostrich an unpromising organ or cell donor for xenotransplantation to man.  (+info)

Lipid-dependent targeting of G proteins into rafts. (5/288)

Domains rich in sphingolipids and cholesterol, or rafts, may organize signal transduction complexes at the plasma membrane. Raft lipids are believed to exist in a state similar to the liquid-ordered phase. It has been proposed that proteins with a high affinity for an ordered lipid environment will preferentially partition into rafts (Melkonian, K. A., Ostermeyer, A. G., Chen, J. Z., Roth, M. G., and Brown, D. A. (1999) J. Biol. Chem. 274, 3910-3917). We investigated the possibility that lipid-lipid interactions between lipid-modified proteins and raft lipids mediate targeting of proteins to these domains. G protein monomers or trimers were reconstituted in liposomes, engineered to mimic raft domains. Assay for partitioning of G proteins into rafts was based on Triton X-100 insolubility. Myristoylation and palmitoylation of Galpha(i) were necessary and sufficient for association with liposomes and partitioning into rafts. Strikingly, the amount of fatty-acylated Galpha(i) in rafts was significantly reduced when myristoylated Galpha(i) was thioacylated with cis-unsaturated fatty acids instead of saturated fatty acids such as palmitate. Prenylated betagamma subunits were excluded from rafts, whether reconstituted alone or with fatty-acylated alpha subunits. These results suggest that the structural difference between lipids that modify proteins is one basis for the selectivity of protein targeting to rafts.  (+info)

Trans interactions between galactosylceramide and cerebroside sulfate across apposed bilayers. (6/288)

The two glycosphingolipids galactosylceramide (GalC) and its sulfated form, cerebroside sulfate (CBS), are present at high concentrations in the multilayered myelin sheath and are involved in carbohydrate-carbohydrate interactions between the lipid headgroups. In order to study the structure of the complex of these two glycolipids by Fourier transform infrared (FTIR) spectroscopy, GalC dispersions were combined with CBS dispersions in the presence and absence of Ca(2+). The FTIR spectra indicated that a strong interaction occurred between these glycolipids even in the absence of Ca(2+). The interaction resulted in dehydration of the sulfate, changes in the intermolecular hydrogen bonding interactions of the sugar and other oxygens, decreased intermolecular hydrogen bonding of the amide C==O of GalC and dehydration of the amide region of one or both of the lipids in the mixture, and disordering of the hydrocarbon chains of both lipids. The spectra also show that Ca(2+) interacts with the sulfate of CBS. Although they do not reveal which other groups of CBS and GalC interact with Ca(2+) or which groups participate in the interaction between the two lipids, they do show that the sulfate is not directly involved in interaction with GalC, since it can still bind to Ca(2+) in the mixture. The interaction between these two lipids could be either a lateral cis interaction in the same bilayer or a trans interaction between apposed bilayers. The type of interaction between the lipids, cis or trans, was investigated using fluorescent and spin-label probes and anti-glycolipid antibodies. The results confirmed a strong interaction between the GalC and the CBS microstructures. They suggested further that this interaction caused the CBS microstructures to be disrupted so that CBS formed a single bilayer around the GalC multilayered microstructures, thus sequestering GalC from the external aqueous phase. Thus the CBS and GalC interacted via a trans interaction across apposed bilayers, which resulted in dehydration of the headgroup and interface region of both lipid bilayers. The strong interaction between these lipids may be involved in stabilization of the myelin sheath.  (+info)

Dimorphic expression of cerebrosides in the mycopathogen Sporothrix schenckii. (7/288)

Major neutral glycosphingolipid components were extracted from Sporothrix schenckii, a dimorphic fungus exhibiting a hyphal saprophytic phase and a yeast parasitic phase responsible for chronic mycotic infections in mammalian hosts. These components, one from the mycelial form and two from the yeast form, were purified and their structures were elucidated by (1)H nuclear magnetic resonance (NMR) spectroscopy, electrospray ionization mass spectrometry (ESI-MS), and tandem ESI-MS/MS. All three were characterized as cerebrosides (monohexosylceramides) containing (4E, 8E)-9-methyl-4,8-sphingadienine as the long-chain base attached to N-2'-hydroxyoctadecanoate and N-2'-hydroxy-(E)-Delta(3)-octadecenoate as the fatty acyl components. However, while the mycelial form expressed only beta-glucopyranosylceramide, the yeast form expressed both beta-gluco- and beta-galactopyranosylceramides in approximately equal amounts. In addition, while the glucosylceramides of both mycelial and yeast forms had similar proportions of saturated and (E)-Delta(3) unsaturated 2-hydroxy fatty acid, the galactocerebroside of the yeast form had significantly higher levels of (E)-Delta(3) unsaturation. The differences in cerebroside hexose structure represent a novel type of glycosphingolipid dimorphism not previously reported in fungi. Possible implications of these findings with respect to regulation of morphological transitions in S. schenckii and other dimorphic fungi are discussed.  (+info)

Exceptionally potent inhibitors of fatty acid amide hydrolase: the enzyme responsible for degradation of endogenous oleamide and anandamide. (8/288)

The development of exceptionally potent inhibitors of fatty acid amide hydrolase (FAAH), the enzyme responsible for the degradation of oleamide (an endogenous sleep-inducing lipid), and anandamide (an endogenous ligand for cannabinoid receptors) is detailed. The inhibitors may serve as useful tools to clarify the role of endogenous oleamide and anandamide and may prove to be useful therapeutic agents for the treatment of sleep disorders or pain. The combination of several features-an optimal C12-C8 chain length, pi-unsaturation introduction at the corresponding arachidonoyl Delta(8,9)/Delta(11,12) and oleoyl Delta(9,10) location, and an alpha-keto N4 oxazolopyridine with incorporation of a second weakly basic nitrogen provided FAAH inhibitors with K(i)s that drop below 200 pM and are 10(2)-10(3) times more potent than the corresponding trifluoromethyl ketones.  (+info)

The properties of bilayers composed of pure brain cerebroside (bCrb) or of binary mixtures of bCrb with brain ceramide, cholesterol, egg phosphatidylcholine or brain sphingomyelin have been studied using a combination of physical techniques. Pure bCrb exhibits a rather narrow gel-fluid transition centred at approximate to 65 degrees C with a half-width at half-height T-1/2 approximate to 3 degrees C. bCrb mixes well with both fluid and gel phospholipids and ceramide, and it rigidifies bilayers of egg phosphatidylcholine or brain sphingomyelin when the latter are in the fluid state. Cholesterol markedly widens the bCrb gel-fluid transition, while decreasing the associated transition enthalpy, in the manner of cholesterol mixtures with saturated phosphatidylcholines, or sphingomyelins. Laurdan and DPH fluorescence indicate the formation of fluid ordered phases in the bCrb:cholesterol mixtures. Macroscopic phase separation of more and less fluid domains is observed in giant unilamellar vesicles ...
SUMMARY: Water-insoluble complexes of ganglioside with cerebroside fixed tetanus toxin at low concentrations (a few LD 50/ml.) of toxin. A complex containing 25% ganglioside with cerebroside was 50 times better at fixing toxin than complexes containing either 2% or 50% ganglioside. A complex containing 25% of a mixture of the gangliosides Giii and Giv was 12 times better at fixing toxin than a similar complex with gangliosides Gi and GII. Complexes of ganglioside with sphingomyelin and lecithin fixed toxin to a slight extent, while complexes with tripalmitin and cholesterol did not fix toxin. The complex of cerebroside and ganglioside, containing 25% ganglioside, did not fix strychnine, serotonin, botulinum toxin or plasma albumin.
Cerebroside sulfatase: …called arylsulfatase A (ASA), or cerebroside sulfatase. Arylsulfatase A deficiency allows certain harmful sulfur-containing lipids, known as sulfosphingolipids (also called sulfatides), to accumulate in nerve tissues of the central nervous system instead of being broken down. Sulfatides can also accumulate in nerve tissue in organs, such as the kidneys and…
TY - JOUR. T1 - Inhibition of in vitro peripheral myelin formation by monoclonal anti-galactocerebroside.. AU - Ranscht, B.. AU - Wood, P. M.. AU - Bunge, R. P.. PY - 1987/9. Y1 - 1987/9. N2 - This work investigates the role of galactocerebroside (GalC) in peripheral myelin formation. A monoclonal antibody against GalC was introduced into a myelinating culture system consisting of rat sensory neurons and Schwann cells, without other cell types. At levels that saturated Schwann cell surface GalC, anti-GalC IgG prevented by more than 99% the appearance of myelin sheaths. Ensheathment and basal lamina deposition were unaffected and many Schwann cells were in the 1:1 relationship that typically develops between Schwann cells and axons prior to myelination. Thus, the anti-GalC antibody did not interfere with the formation of the mesaxon but prevented its elongation. When experimentally restrained from myelination, Schwann cells did not accumulate the myelin proteins PO and basic protein; only low ...
Although the CD4 molecule is the principal cellular receptor for the human immunodeficiency virus (HIV), several CD4-negative cell lines are susceptible to infection with one or more HIV strains. These findings indicate that there are alternate modes of viral entry, perhaps involving one or more receptor molecules. Antibodies against galactosyl ceramide (galactocerebroside, or GalC) inhibited viral internalization and infection in two CD4-negative cell lines derived from the nervous system: U373-MG and SK-N-MC. Furthermore, recombinant HIV surface glycoprotein gp120 bound to GalC but not to other glycolipids. These results suggest a role for GalC or a highly related molecule in HIV entry into neural cells. ...
lactosylceramidase: ceramide-Glc-Gal & H2O gives Cer-Glc & galactose; deficiency gives lactosylceramidosis; see also lactosylceramidase II which acts GM(1) ganglioside
The root sphingo- comes from the Greek word sphingein which means to to hold fast. There are 12 different entries containing this root. glycosphingolipid- any of various lipids (such as a cerebroside or a ganglioside) sphingometer- an instrument for measuring the bending of a strut (as by deflection of beams of light) sphingomyelin- any of…
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1. The lipids of whole brain and subcellular fractions of the rat were analysed during development. 2. The deposition of cholesterol occurred in two phases, one related to increasing wet weight of the brain and the second to myelination. Cerebroside accumulation was related only to myelination. 3. The composition of myelin isolated from 12-day-old rat brain was different in some respects from that of the adult. In the former there was an increase of phospholipid in relation to cholesterol and a marked deficiency in cerebroside. 4. It is suggested that early myelin is extruded glial plasma membrane, which only later becomes mature myelin.. ...
At the foundation of embalming is formalin, an aqueous solution saturated with formaldehyde gas, which constitutes 40% by volume and 37% by weight of the solution. Formaldehyde coagulates protoplasmic protein, turning it from a soft soluble form to a hard insoluble form which is resistant to both autolytic and bacterial proteolytic enzymes. Moreover, formaldehyde kills bacteria by coagulating bacterial protoplasm and has a drying effect upon tissue because of its strong affinity for water. Because formaldehyde preserves lipids as well as proteins, it would seem to be especially of value for the brain. A study of human brains preserved in formaldehyde for up to 24 years showed that the preservation of lipids was not uniform: cholesterol, cerebrosides, sulphatides, phosphoinositides and sphingomyelin remained unaffected, whereas lecithin, phosphatidylethanolamine and phosphadidylserine were broken down [THE JOURNAL OF HISTOCHEMISTRY AND CYTOCHEMISTRY; 10:704-709 (1962)]. Because unbuffered ...
NPGS is the top selling SEM lithography system at research institutions in North America, and its use is becoming widespread around the world. The objective for NPGS is to provide a powerful ...
article{8f49d55d-b5f5-4f7b-bd69-f3ec250ab558, abstract = {Intercellular and covalently bound lipids within the stratum corneum (SC), the outermost layer of the epidermis, are the primary barrier to cutaneous water loss (CWL) in birds. We compared CWL and intercellular SC lipid composition in 20 species of birds from desert and mesic environments. Furthermore, we compared covalently bound lipids with CWL and intercellular lipids in the lark family (Alaudidae). We found that CWL increases in birds from more mesic environments, and this increase was related to changes in intercellular SC lipid composition. The most consistent pattern that emerged was a decrease in the relative amount of cerebrosides as CWL increased, a pattern that is counterintuitive based on studies of mammals with Gaucher disease. Although covalently bound lipids in larks did not correlate with CWL, we found that covalently bound cerebrosides correlated positively with intercellular cerebrosides and intercellular cholesterol ...
Extraction of control human spleen glucocerebrosidase with sodium cholate and butan-l-ol reversibly inactivates the enzyme in terms of its ability to hydrolyse the water-soluble substrate 4-methylumbelliferyl beta-D-glucopyranoside (MUGlc). The acidic brain lipid galactocerebroside 3-sulphate (sulphatide) reconstitutes beta-glucosidase activity in a strongly concentration-dependent manner. In this study we show that sulphatide exhibits three critical micellar concentrations (CMCs): CMC1, 3.72 microM; CMC2, 22.6 microM; CMC3, 60.7 microM. We designate the aggregates formed at these CMCs as primary, secondary and tertiary micelles respectively. From the results of kinetic studies performed at various sulphatide concentrations (0.012-248 microM), we found that sulphatide monomers (less than 3 microM) decreased the Km (for MUGlc) of control glucocerebrosidase from 11 to 4.6 mM, and lowered the Vmax. 2-fold. However, secondary and tertiary micelles were required for expression of high control ...
Metachromatic Leukodystrophy (MLD) is a rare autosomal recessive disorder caused by the deficiency of the Arylsulfatase A enzyme (ARSA), resulting in accumulation of galactosyl sulfatide (cerebroside sulfate), a major constituent of the myelin sheath. Accumulation of sulfatides leads to a progressive degeneration of the white matter in the central and peripheral nervous systems (CNS, PNS) and to a neuronal degeneration. The late-infantile form of MLD, which is usually diagnosed in the second year of life, is the most frequent and severe form of the disease. The prognosis is severe, leading to vegetative stage or death within few years after the diagnosis. There is no treatment for patients affected with this early-onset form of the disease.. Conventional MRI (1.5 Tesla) shows extensive involvement of the cerebral white matter (hypo-T1, hyper- T2 and FLAIR signals) indicative of rapidly progressing leukodystrophy. Early cortical atrophy reflects associated neuronal involvement. Proton MR ...
Metachromatic leukodystrophy (MLD) is a lysosomal storage disorder caused by a deficiency of the arylsulfatase A (ARSA) enzyme, which leads to the accumulation of galactosyl sulfatide (cerebroside sulfate) in the white matter of the central nervous system and in the peripheral nervous system. Galactosyl sulfatide and, to a smaller extent, lactosyl sulfatide, also accumulate within the kidney, gallbladder, and other visceral organs and are excreted in excessive amounts in the urine.. The 3 clinical forms of MLD are late-infantile, juvenile, and adult, depending on age of onset. All result in progressive neurologic changes and leukodystrophy demonstrated on magnetic resonance imaging. Late-infantile MLD is the most common (50%-60% of cases) and usually presents between age 1 to 2 years with hypotonia, clumsiness, diminished reflexes, and slurred speech. Progressive neurodegeneration occurs and most patients die within 5 years of the diagnosis. Juvenile MLD (20%-30% of cases) is characterized by ...
Glucocerebroside - A type of fat (lipid) molecule accumulates in individuals with Gaucher disease and is used as a building block to make certain cell membrane...
Phospholipids and cerebrosides of the normal chick sciatic nerve were extracted by Folch procedures and separated with silica gel thin-layer chromatography using various chloroform-methanol solvents. The subsequent chemical assays were expressed as a function of the developmental stages of the chick from 15 days of incubation to 7 days after hatching (28 days from onset of incubation). A histological study of chick sciatic nerves at ages 18, 20, 22 and 25 days was conducted to identify the period of myelination. Luxol Fast Blue G stain was used to identify the myelin. Myelin, is poorly identified at 18 days but notably present at 25 days. The assays of the phospholipids, expressed as relative percent of the total phospholipid phosphorus, indicated that ethanolamine-phosphoglyceride ratio increases significantly from 17 days to 23 days from onset. Sphingomyelin ratio also increased during this time, but not as much. While the relative amount of serinephosphoglyceride remained constant and ...
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Acer3 upregulation is important for the homeostasis of complex sphingolipid in aging brain.A and B. Levels of individual monohexosylceramide (HexCer) species an
OGT2378 is a GCS inhibitor (Glucosyl Ceramide Synthase Inhibitor) with potential anticancer activity. In murine models with melanoma, OGT2378 decreased tumor size, and showed no cytotoxic and apoptotic effects on MEB-4 melanomacells. Additionally, it has been reported that OGT2378 is biologically active, and a well tolerated agent in in vivo system.
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Metachromatic Leukodystrophy (MLD) is a rare autosomal recessive disorder caused by the deficiency of the Arylsulfatase A enzyme (ARSA), resulting in accumulation of galactosyl sulfatide (cerebroside sulfate), a major constituent of the myelin sheath. Accumulation of galactosyl sulfatides leads to a progressive degeneration of the white matter in the central and peripheral nervous system (CNS, PNS) and neuronal degeneration. The late infantile form of MLD, which usually is diagnosed in the second year of life, is the most frequent and severe form of the disease. The prognosis is severe, leading to vegetative stage or death within few years after the diagnosis. There is no treatment for patients affected with this early onset form of the disease. In patients with late-onset MLD (juvenile and adult forms), allogeneic hematopoietic stem cell transplantation can stabilize the cerebral demyelination. This treatment is however inefficient in patients with late infantile MLD at a symptomatic stage. The ...
Gauchers disease [DOID:1926]. A sphingolipidosis characterized by deficiency of the enzyme glucocerebrosidase which results in the accumulation of harmful quantities of the glycolipid glucocerebroside throughout the body, especially within the bone marrow, spleen and liver.. Synonyms: Gauchers disease, DOID:1926, Gauchers disease, Gauchers disorder, Gauchers syndrome .... Linkouts: OMIM #1 #2 #3 #4 #5 #6. ...
Sphingolipids, or glycosylceramides, are a class of lipids containing a backbone of sphingoid bases, a set of aliphatic amino alcohols that includes sphingosine. They were discovered in brain extracts in the 1870s and were named after the mythological Sphinx because of their enigmatic nature. These compounds play important roles in signal transmission and cell recognition. Sphingolipidoses, or disorders of sphingolipid metabolism, have particular impact on neural tissue. A sphingolipid with an R group consisting of a hydrogen atom only is a ceramide. Other common R groups include phosphocholine, yielding a sphingomyelin, and various sugar monomers or dimers, yielding cerebrosides and globosides, respectively. Cerebrosides and globosides are collectively known as glycosphingolipids. The long-chain bases, sometimes simply known as sphingoid bases, are the first non-transient products of de novo sphingolipid synthesis in both yeast and mammals. These compounds, specifically known as ...
Arylsulfatase A (or cerebroside-sulfatase) is an enzyme that breaks down sulfatides, namely cerebroside 3-sulfate into cerebroside and sulfate. In humans, arylsulfatase A is encoded by the ARSA gene. A deficiency is associated with metachromatic leukodystrophy, an autosomal recessive disease. Arylsulfatase A is inhibited by phosphate, which forms a covalent bond with the active site 3-oxoalanine. GRCh38: Ensembl release 89: ENSG00000100299 - Ensembl, May 2017 GRCm38: Ensembl release 89: ENSMUSG00000022620 - Ensembl, May 2017 Human PubMed Reference:. Mouse PubMed Reference:. Stein C, Gieselmann V, Kreysing J, Schmidt B, Pohlmann R, Waheed A, Meyer HE, OBrien JS, von Figura K (January 1989). Cloning and expression of human arylsulfatase A. J. Biol. Chem. 264 (2): 1252-9. PMID 2562955. Matzner U, Herbst E, Hedayati KK, Lüllmann-Rauch R, Wessig C, Schröder S, Eistrup C, Möller C, Fogh J, Gieselmann V (May 2005). Enzyme replacement improves nervous system pathology and function in a mouse ...
Causes:. A deficit of the enzyme glucocerebrosidase is the cause of Gauchers disease. Normally, this enzyme breaks down glucocerebrosides, which are fatty substances (lipids). However, these fatty substances could build up in someones brain and other organs and within the bone marrow in case the enzyme is scarce. Autosomal recessive is the inheritance pattern via which Gauchers disease is passed along. In order for a child to have the condition, both parents have to be carriers of a Gauchers genetic mutation. So far, more than 300 genetic mutations have been associated with this disease. Theres still only a 25% chance that the child will develop the disease even when both parents are carriers. Theres also a 25% chance that the child will not be a carrier and not have the disease, and a 50% chance that the child will be an unaffected carrier ...
Types A and B appear most often in Jewish families. Type C affects all ethnic groups and is the most common. Ataxia and dystonia are followed by supranuclear vertical gaze palsy, seizures, and dementia. Hepatosplenomegaly often coexists. Foamy (lipid-laden) cells or sea-blue histiocytes in the liver and bone marrow are diagnostic. Metachromatic Leukodystrophy (Arylsulfatase A or Saposin B Deficiency) Deficiency of arylsulfatase A or its activator, saposin B, leads to accumulation of cerebroside sulfate, which causes progressive (frontal-predominant) central and peripheral demyelination. ADHD affects about 5% of school-aged children worldwide, predominantly males (3:1 to 8:1). About one-third of ADHD cases have at least one ADHD parent. The risk to first-degree relatives of affected individual is 8-10 times that of the general population. ADHD persists into adolescence in about 30%-50% of affected individuals. The DSM-IV-TR distinguishes two dimensions in the behavior of individuals with ADHD: ...
Arylsulfatase A (ASA) is a lysosomal enzyme involved in the catabolism of cerebroside sulfate. ASA deficiency is associated with metachromatic leukodystrophy (MLD). Low ASA activities have also been reported in a more common condition with no apparent clinical consequences termed ASA pseudo-deficiency (ASA-PD) which is associated with two linked mutations in the ASA gene (c.1049A | G and c.*96A | G). This study aimed to investigate the frequency of the two ASA-PD variants and their linkage disequilibrium (LD) among Tunisians. ASA-PD variants were detected in 129 healthy Tunisians and their frequencies were compared to those described worldwide. The frequency of the PD allele was estimated at 17.4 % for the overall sample, with c.1049A | G and c.*96A | G frequencies of 25.6 and 17.4 %, respectively. This study also revealed a high LD between the two ASA-PD variants (r (2) = 0.61). Inter-population analysis revealed similarities in the ASA-PD genetic structure between Tunisians and populations from Middle
Looking for online definition of galactosylceramide in the Medical Dictionary? galactosylceramide explanation free. What is galactosylceramide? Meaning of galactosylceramide medical term. What does galactosylceramide mean?
Gauchers Disease* This disease was first described in the year 1882 by a French physician, Dr. Philippe C.E. Gaucher. As per him this disease or disorder takes place due to Accumulation of a particular lipid. Gaucher disease is basically defined as an inherited metabolic disorder (caused due to defective gene for the enzyme glucocerebrosidase from both parents) that leads to the accumulation of a particular fatty substance through out the body. Due to the accumulation of lipids in the body, it results in to deficiency of a particular enzyme acid beta-glucocerebrosidase which is necessary for the breakdown of a particular lipid- glucosyl ceramide. As the lipid accumulates, the glucosyl ceramide gets stored in macrophages (the scavenges cells of the body), which takes up characteristic appearance unique to those affected with this disorder (Gaucher Cells - basically termed as). These lipids get accumulated in the spleen, liver, bone marrow, brain and the lungs. Basically there are three types of ...
Although previous reports have demonstrated that dietary glucosylceramide can decrease TEWL from human skin [24], the mechanism was unclear. Ueda et al. reported that orally administrated ceramide was distributed to the dermis after intestinal absorption, followed by transfer from the dermis to the epidermis [25]. Ishikawa et al. also showed that dietary glucosylceramide was degraded into sphingoids, which were absorbed through the intestinal epithelial cells of rats and were subsequently also found in the lymph fluid in these rats [11]. Another study showed that 4-hydroxysphinganine could activate the PPARs [26] that are the key regulators of keratinocyte differentiation [27].. Keratinocyte differentiation is closely related to internal ceramide synthesis [28]. It is unlikely that orally ingested glucosylceramide would specifically localize to the skin because the amount of ingested glucosylceramide is simply too little to enhance ceramide levels in the skin. We hypothesized that sphingoids are ...
Facilitates the transfer of a spectrum of different lipid molecules, including diacylglycerol, phosphatidic acid, sphingomyelin, phosphatidylcholine, phosphatidylglycerol, cerebroside and phosphatidyl ethanolamine. Essential for the transfer of excess surface lipids from triglyceride-rich lipoproteins to HDL, thereby facilitating the formation of smaller lipoprotein remnants, contributing to the formation of LDL, and assisting in the maturation of HDL particles. PLTP also plays a key role in the uptake of cholesterol from peripheral cells and tissues that is subsequently transported to the liver for degradation and excretion. Two distinct forms of PLTP exist in plasma: an active form that can transfer PC from phospholipid vesicles to high-density lipoproteins (HDL), and an inactive form that lacks this capability.
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Detailed step-by-step protocols for all procedures are available on request (barres{at}stanford.edu).. Reagents. Recombinant human trophic factors were obtained from Peprotech (bFGF), Cambridge Neuroscience (GGF2), Regeneron (CNTF), and R & D Systems (Minneapolis, MN; LIF). Monoclonal antibodies were obtained from Serotec (Indianapolis, IN; MRC-OX7 anti-Thy1.1 IgG antibody), American Type Culture Collection (Rockville, MD; A2B5), Jim Cohen (C5 neuroepithelial antibody), Developmental Hybridoma Bank (RAT401 anti-nestin antibody), Sigma (St. Louis, MO; anti-S100β antibody), Barbara Ranscht [Rmab anti-galactocerebroside (anti-GC) antibody], Ursula Drager (R5 anti-vimentin antibody), and Boehringer Mannheim [Indianapolis, IN; anti-5-bromo-2′-deoxyuridine (BrdU) antibody]. Polyclonal antibodies were obtained from Babco (Richmond, CA; rabbit anti-Pax2 antiserum) and Dako (Carpinteria, CA; rabbit anti-GFAP antiserum).. Preparation of optic nerve cell suspension. Sprague Dawley rats (Simonson Labs) ...
Novel, optically active azido alcohols are synthesized from N-haloacetyl oxazolidinone and are coupled with protected, activated carbohydrate residues in stereoselective glycosidic fashion to provide glycolipids in substantially pure stereochemical form. In accordance with certain preferred embodiments, the glycosphingolipids globotriaosylceramide, lactosyl ceramide, and galactosyl ceramide are synthesized in substantially pure stereochemical form from alkenyl, C-18 azido alcohols and protected fluoroglycosides and are employed as antigens in the production of antibodies useful, for example, in the treatment of disease.
Involved in the production of sphingolipid metabolites. Active on sphingosine, phytosphingosine (PHS, 4-hydroxysphinganine), D-erythro-dihydrosphingosine, D-erythro-sphingosine and trans-4, trans-8-sphingadienine, an LCB found exclusively in plants, but not on N-acetyl-dihydrosphingosine (C2-dihydroceramide) and D-threo-dihydrosphingosine.
Summary The study of Gauchers disease-Market Insights, Epidemiology and Market Forecast-2023-United States is very important to enhance business producti
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Lamkin, W.M.; Weatherford, J.W.; Jones, N.S.; Pan, T.; Ward, D.N., Analysis of methylthiohydantoines of amino acids by gas-liquid chromatography of their trimethylsilyl derivatives, Anal. Biochem., 1974, 58, 2, 422-438, https://doi.org/10.1016/0003-2697(74)90211-5 . 19 matching species were found. For each matching species the following will be displayed: ...
Definition of globoid cell in the Financial Dictionary - by Free online English dictionary and encyclopedia. What is globoid cell? Meaning of globoid cell as a finance term. What does globoid cell mean in finance?
Synonyms for Acute cerebral Gaucher's disease in Free Thesaurus. Antonyms for Acute cerebral Gaucher's disease. 3 words related to Gauchers disease: monogenic disease, monogenic disorder, lipidosis. What are synonyms for Acute cerebral Gaucher's disease?
Sphingosine-1-phosphate and its receptors may be involved in vascular smooth muscle cell (VSMC) proliferation following vascular injury. Here, we evaluate the effect of d-erythro-N,N-dimethylsphingosine (DMS), a sphingosine kinase (SK) inhibitor, on VSMC proliferation, apoptosis and neointimal formation. Growth responses in vitro to fetal calf serum (FCS) were measured by [3H]-thymidine incorporation and extracellular signal-regulated kinase-1/2 (ERK-1/2) activation in quiescent primary cultures of porcine VSMC in the presence and absence of various concentrations of the SK inhibitor DMS. In vivo treatment with DMS was delivered with a local endoluminal catheter, following balloon injury of coronary arteries. The artery intimal formation was investigated by angiography, myography and histomorphometry. In vitro experiments indicated that DMS induced a dose-dependent reduction in [3H]-thymidine incorporation and ERK-1/2 activation via a protein kinase C (PKC) independent mechanism with an IC50 ...
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The mechanism of membrane domain formation was investigated through analysis of domains induced both by calcium and myelin basic protein in vesicles containing components normally enriched in myelin. In the presence of calcium or myelin basic protein, domains enriched in phosphatidic acid, phosphatidylserine, galactocerebroside, and sulfatide were observed to form. In these domains phosphatidic acid or phosphatidylserine co-localized with galactocerebroside and sulfatide. Sphingomyelin was unable to partition into a domain unless sulfatide was also present ...
Gaucher (pronounced go-SHAY) disease is an inherited illness caused by a gene mutation. Normally, this gene is responsible for an enzyme called glucocerebrosidase that the body needs to break down a particular kind of fat called glucocerebroside. In people with Gaucher disease, the body is not able to properly produce this enzyme and the fat cannot be broken down. It then accumulates, mostly in the liver, spleen and bone marrow.
TY - JOUR. T1 - Differential unmasking of adenylate cyclase activity (AC) in cardiac membrane sheets and vesicles. AU - Fleming, J. W.. AU - Besch, H. R.. AU - Jones, L. R.. AU - Watanabe, A. M.. PY - 1978/1/1. Y1 - 1978/1/1. UR - http://www.scopus.com/inward/record.url?scp=0017841068&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=0017841068&partnerID=8YFLogxK. M3 - Article. AN - SCOPUS:0017841068. VL - 20. JO - Pharmacologist. JF - Pharmacologist. SN - 0031-7004. IS - 3. ER - ...
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WASHINGTON -- A second company has been given an FDA go-ahead to market an as-yet-unapproved drug to treat Gauchers disease, helping to remedy a shortage of the only agent approved for the condition.
Rouser, G., G. Kritchevsky, A. N. Siakotos and A. Yamamoto, in An Introduction to Neuropathology: Methods and Diagnosis, C. G. Tedeschi, editor, Little, Brown and Co., in press.Google Scholar ...
Page contains details about β-galactosidase/ZIF-8 MOF coating . It has composition images, properties, Characterization methods, synthesis, applications and reference articles : nano.nature.com
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Cerebrosides have a single glucose or galactose at the 1-hydroxy position. Sulfatides are sulfated cerebrosides. Gangliosides ... yielding cerebrosides and globosides, respectively. Cerebrosides and globosides are collectively known as glycosphingolipids. ...
1. Imaging of Cerebrosides Directly from Rat Brain Tissue". Analytical Chemistry. 79 (6): 2373-85. doi:10.1021/ac062251h. PMID ...
Fleischer B, Zambrano F (1973). "Localization of cerebroside-sulfotransferase activity in the Golgi apparatus of rat kidney". ... Benjamins JA, Hadden T, Skoff RP (1982). "Cerebroside sulfotransferase in Golgi-enriched fractions from rat brain". J. ... Farrell DF, McKhann GM (1971). "Characterization of cerebroside sulfotransferase from rat brain". J. Biol. Chem. 246 (15): 4694 ...
... s A-H are neuritogenic cerebrosides isolated from the mushroom Termitomyces albuminosus. Termitomycesphins G ... and H, additional cerebrosides from the edible Chinese mushroom Termitomyces albuminosus. ...
His proposals on the biosynthesis and degradation of cerebroside-3-sulfate, a lipid found in high concentrations in patients ... Bimal K Bachhawat; James Austin; Donald Armstrong (1967). "A Cerebroside Sulphotransferase Deficiency in a Human Disorder of ... Farooqui, Bachhawat B. K. (1973). "Enzymic desulfation of cerebroside-3'-sulfate by chicken brain arylsulfatase". J. Neurochem ... B. K. (1965). "Formation of cerebroside sulfate from 3'-phosphoadenosine-5'phosphosulfate in sheep brain". Biochim. Biophys. ...
It is found in wood tar, various cerebrosides, and in small amounts in most natural fats. The fatty acids of peanut oil contain ...
... (also called glucosylceramide) is any of the cerebrosides in which the monosaccharide head group is glucose. ...
The simplest glycolipid is cerebroside, in which there is only one sugar residue, either Glc or Gal. More complex glycolipids, ...
At this time, [35S] Sulfate was incorporated into sulfatide and the activity of cerebroside, sulfotransferase reached a peak in ... In conclusion, the early phase of myelination was correlated with the increases synthesis of lipids, cholesterol, cerebroside, ... cerebroside, and sulfatide. As researchers began to do postnatal research, they found that myelinogenesis in the rat optic ...
A galactosylceramide, or galactocerebroside is a type of cerebroside consisting of a ceramide with a galactose residue at the 1 ...
... s include: Cerebrosides Gangliosides Globosides Gangliosides are mainly found in the cell membranes of the ... In the case of cerebrirosis, this affects the cerebrosides (examples are Gaucher's disease and Krabbe's disease), in case of ...
... cerebrosides MeSH D09.400.410.420.525.200.250.450 - galactosylceramides MeSH D09.400.410.420.525.200.250.490 - ...
Cerebrosides (cells from the nervous systems of cattle or swine) were once used in some high-end skin-care products to increase ...
... cerebrosides MeSH D10.390.470.675.200.250.450 - galactosylceramides MeSH D10.390.470.675.200.250.490 - glucosylceramides MeSH ... cerebrosides MeSH D10.570.877.360.612.200.250.450 - galactosylceramides MeSH D10.570.877.360.612.200.250.490 - ...
This acid is an important member of the group of the cerebrosides, which are fatty acids of the glycosphingolipids group, ...
... cerebroside-sulfatase EC 3.1.6.9: chondro-4-sulfatase EC 3.1.6.10: chondro-6-sulfatase EC 3.1.6.11: disulfoglucosamine-6- ...
"Fatty acid composition of cerebrosides and cerebroside sulphatides in cerebral oedema". Acta Neuropathologica. 12 (1): 62-67. ... Cerebroside molecules are able form up to eight intermolecular hydrogen bonds between the polar hydrogens of the sugar and the ... Cerebrosides containing a sulfuric ester (sulfate) group, known as sulfatides, also occur in the myelin sheath of nerves. These ... Cerebroside synthesis can therefore give a measurement of myelin formation or remyelination. The sugar moiety is linked ...
Defects in the enzyme alpha-galactosidase lead to the buildup of globotriaosylceramide, causing Fabry's disease.[3] The pharmaceutical drug migalastat enhances the function of alpha-galactosidase and is used to treat Fabry's. Globotriaosylceramide is also one of the targets of Shiga toxin, which is responsible for pathogenicity of enterohemorrhagic Escherichia coli (EHEC). The bacterial Shiga toxin can be used for targeted therapy of gastric cancer, because this tumor entity expresses the receptor of the Shiga toxin. For this purpose an unspecific chemotherapeutical is conjugated to the B-subunit to make it specific. In this way only the tumor cells, but not healthy cells should be destroyed during therapy.[4] ...
Examples of these are the simple and complex glycosphingolipids such as cerebrosides and gangliosides. ...
Plasma membranes also contain carbohydrates, predominantly glycoproteins, but with some glycolipids (cerebrosides and ...
"Fatty acid composition of cerebrosides and cerebroside sulphatides in cerebral oedema". Acta Neuropathologica. 12 (1): 62-67. ... Cerebroside molecules are able form up to eight intermolecular hydrogen bonds between the polar hydrogens of the sugar and the ... Cerebrosides containing a sulfuric ester (sulfate) group, known as sulfatides, also occur in the myelin sheath of nerves. These ... Cerebroside synthesis can therefore give a measurement of myelin formation or remyelination. The sugar moiety is linked ...
cerebroside* Any one of a class of glycolipids [1] in which a single sugar unit is bound to a sphingolipid (see phospholipid [2 ... cerebroside Any one of a class of glycolipids in which a single sugar unit is bound to a sphingolipid (see phospholipid). The ... cerebrosides A Dictionary of Food and Nutrition © A Dictionary of Food and Nutrition 2005, originally published by Oxford ... cerebroside A Dictionary of Biology © A Dictionary of Biology 2004, originally published by Oxford University Press 2004. ...
... is a ceramide (CHEBI:17761) cerebroside (CHEBI:23079) is a neutral glycosphingolipid (CHEBI:25513) ... HexCer(d37:2) (CHEBI:138902) is a cerebroside (CHEBI:23079). hexosyl-(1↔1ʼ)-N-acylsphingosine (CHEBI:82917) is a cerebroside ( ... HexCer(d32:1) (CHEBI:138900) is a cerebroside (CHEBI:23079). HexCer(d33:2) (CHEBI:138901) is a cerebroside (CHEBI:23079). ... N-tetracosanoyl hexosylceramide (CHEBI:77463) is a cerebroside (CHEBI:23079). galactosylceramide (CHEBI:36498) is a cerebroside ...
... or cerebroside sulfatase. Arylsulfatase A deficiency allows certain harmful sulfur-containing lipids, known as ... called arylsulfatase A (ASA), or cerebroside sulfatase. Arylsulfatase A deficiency allows certain harmful sulfur-containing ...
cerebroside synonyms, cerebroside pronunciation, cerebroside translation, English dictionary definition of cerebroside. n. Any ... cerebroside. Also found in: Medical, Encyclopedia, Wikipedia.. Related to cerebroside: ganglioside. cer·e·bro·side. (sĕr′ə-brə- ... IFB-121 * Cerebroside 1 * Cerebroside 2 Phornopsis sp.. Endophytic fungi--the treasure chest of antibacterial substances ... Cerebroside - definition of cerebroside by The Free Dictionary https://www.thefreedictionary.com/cerebroside ...
What is cerebroside-sulfatase? Meaning of cerebroside-sulfatase medical term. What does cerebroside-sulfatase mean? ... Looking for online definition of cerebroside-sulfatase in the Medical Dictionary? cerebroside-sulfatase explanation free. ... cerebroside-sulfatase. cer·e·bro·side-sul·fa·tase. , cerebroside sulfatidase. an enzyme that cleaves sulfate from a sulfated ... A gene on chromosome 22q13.31-qter that encodes arylsulfatase A, which hydrolyses cerebroside sulfate to cerebroside and ...
... ceramide beta-D-galactoside This cerebroside product is a glycosphingolipid containing a galactose (galactocerebroside) ... Scientific Name:Cerebrosides, bovine. *Common Name:Galactosylceramide; Ceramide beta-D-galactoside (contains both hydroxy and ... This cerebroside product is a glycosphingolipid containing a galactose (galactocerebroside) attached to a ceramide acylated ... This leads to an accumulation of cerebroside and psychosine (which is very cytotoxic and can result in demyelination of nerves ...
Cerebroside antibody binding activity was only partially blocked by 0.5 M galactose or α- and β-methylgalactopyranoside, ... Abstract A natural cerebroside (antiC) IgM antibody was found at relatively high levels in the serum of every healthy ... A natural cerebroside (antiC) IgM antibody was found at relatively high levels in the serum of every healthy individual studied ... Cerebroside antibody binding activity was only partially blocked by 0.5 M galactose or α- and β-methylgalactopyranoside, ...
The present study was undertaken to explore the preventive effect of dietary sea cucumber cerebroside (SCC) extracted from ... And the cerebroside was also reported to regulate lipid levels according to Cohn Jeffrey [24]. The marine cerebroside displayed ... Dietary sea cucumber cerebroside alleviates orotic acid-induced excess hepatic adipopexis in rats. *Bei Zhang1. , ... Gao Z, Zhou X, Hu XQ, Xue CH, Xu J, Wang YM: Effects of sea cucumber cerebroside and its long-chain base on lipid and glucose ...
The properties of bilayers composed of pure brain cerebroside (bCrb) or of binary mixtures of bCrb with brain ceramide, ...
Structure of Cerebrosides. II. Small Angle X-Ray Diffraction Study of Cerasine. Hosemann, R. / Loboda-Čačković, J. / Čačković, ...
... cerebroside explanation free. What is cerebroside? Meaning of cerebroside medical term. What does cerebroside mean? ... Looking for online definition of cerebroside in the Medical Dictionary? ... cerebroside. Also found in: Dictionary, Encyclopedia, Wikipedia.. Related to cerebroside: ganglioside. cerebroside. [sĕ-re´bro- ... The sulfate esters of cerebrosides are among the sulfatidates.. cerebroside. /cer·e·bro·side/ (ser´ĕ-bro-sīd″) a general ...
Mitteilung über Cerebroside. Klenk, E. / Leupold, F. Page 208. Published Online: 10/15/2009 ...
Cerebrosides. Targeting of lactosylceramide-containing liposomes to hepatocytes in vivo. (1983) Biochem. Biophys. Acta Volume ...
Cerebrosides. 0.5. Cholesterol. 0.5. Source: Principles of Meat Science by Forrest et al. (1975) W.H. Freeman & Co. ...
Cerebrosides have a single glucose or galactose at the 1-hydroxy position. Sulfatides are sulfated cerebrosides. Gangliosides ... yielding cerebrosides and globosides, respectively. Cerebrosides and globosides are collectively known as glycosphingolipids. ...
... cerebrosides; dicetylphosphate; dipalmitoylphosphatidylglycerol; stearylamine; dodecylamine; hexadecyl-amine; acetyl palmitate ...
... cerebrosides; (4) PE; (5) PC; (6) phosphatidylserine plus phosphatidylinositol.O, Origin. ...
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Cerebroside-Sulfatase. *MLD. *sulfatidase. Additional Information & Resources. Tests Listed in the Genetic Testing Registry. * ...
Vesicular transport of myelin proteolipid and cerebroside sulfates to the myelin membrane. J. Neurosci. Res. 35, 402 - 408. * ... and its sulfated form cerebroside sulfate (CBS), and cholesterol (Norton 1977). It has a complex structure as it contains ...
Fingerprint Dive into the research topics of A model system for opiate-receptor interactions: Mechanism of opiate-cerebroside ... A model system for opiate-receptor interactions: Mechanism of opiate-cerebroside sulfate interaction. ...
glucosyl cerebroside lipidosis. *glucosylceramidase deficiency. *glucosylceramide beta-glucosidase deficiency. * ...
glucosyl cerebroside lipidosis. *glucosylceramidase deficiency. *glucosylceramide beta-glucosidase deficiency. * ...
8.4 Cerebroside, 205. 8.4.1 Positive-Ion Mode, 205. 8.4.2 Negative-Ion Mode, 207. 8.5 Sulfatide, 208. 8.6 Oligoglycosylceramide ...
8.4 Cerebroside, 205. 8.4.1 Positive-Ion Mode, 205. 8.4.2 Negative-Ion Mode, 207. 8.5 Sulfatide, 208. 8.6 Oligoglycosylceramide ...
... one cerebroside, one anthraquinone, and four others. When considering relative abundance, alkaloids accounted for 32.74%, fatty ...
The complex of cerebroside and ganglioside, containing 25% ganglioside, did not fix strychnine, serotonin, botulinum toxin or ... A complex containing 25% ganglioside with cerebroside was 50 times better at fixing toxin than complexes containing either 2% ... Water-insoluble complexes of ganglioside with cerebroside fixed tetanus toxin at low concentrations (a few LD 50/ml.) of toxin ... The Effect of Cerebroside and Other Lipids on the Fixation of Tetanus Toxin by Gangliosides * W. E. van Heyningen and Jane ...
  • cerebrosides Complex lipids, containing the carbohydrate galactose , important in the myelin sheath of nerves. (encyclopedia.com)
  • This enzyme catalyses the following chemical reaction a cerebroside 3-sulfate + H2O ⇌ {\displaystyle \rightleftharpoons } a cerebroside + sulfate This enzyme hydrolyses galactose-3-sulfate residues in a number of lipids. (wikipedia.org)
  • any of a class of lipids , comprising the cerebrosides and gangliosides, that upon hydrolysis yield galactose or a similar sugar, a fatty acid, and sphingosine or dihydrosphingosine. (dictionary.com)
  • Cerebrosides containing a sulfuric ester (sulfate) group, known as sulfatides, also occur in the myelin sheath of nerves. (wikipedia.org)
  • Cerebroside-sulfatase (EC 3.1.6.8, arylsulfatase A, cerebroside sulfate sulfatase) is an enzyme with systematic name cerebroside-3-sulfate 3-sulfohydrolase. (wikipedia.org)
  • A gene on chromosome 22q13.31-qter that encodes arylsulfatase A, which hydrolyses cerebroside sulfate to cerebroside and sulfate. (thefreedictionary.com)
  • The sulfate esters of cerebrosides are among the sulfatidates. (thefreedictionary.com)
  • Existence of Cerebroside and Cholesterol Sulfate in the Tissues of the Sea Star Asterias rubens . (springer.com)
  • On cerebrosides and gangliosides. (bris.ac.uk)
  • Glycosphingolipids may be further subdivided into cerebrosides and gangliosides. (humpath.com)
  • Cerebrosides have a single glucose or galactose at the 1-hydroxy position, while gangliosides have at least three sugars, one of which must be sialic acid. (humpath.com)
  • Cerebroside molecules are able form up to eight intermolecular hydrogen bonds between the polar hydrogens of the sugar and the hydroxy and amide groups of the sphingosine base of the ceramide. (wikipedia.org)
  • Cerebrosides are an example which contain the sphingosine backbone attached to a fatty acid and a carbohydrate. (libretexts.org)
  • Phytochemical studies of EB have revealed the presence of various compounds like coumarin, [beta]-sitosterol, cerebroside , ceramide and quercetin 3-o-rutinoside. (thefreedictionary.com)
  • The fundamental structure of a cerebroside is ceramide. (wikipedia.org)
  • Monoglycosyl and oligoglycosylceramides having a mono or polysaccharide bonded glycosidically to the terminal OH group of ceramide are defined as cerebrosides. (wikipedia.org)
  • This cerebroside product is a glycosphingolipid containing a galactose (galactocerebroside) attached to a ceramide acylated with hydroxy and non-hydroxy fatty acids. (matreya.com)
  • The properties of bilayers composed of pure brain cerebroside (bCrb) or of binary mixtures of bCrb with brain ceramide, cholesterol, egg phosphatidylcholine or brain sphingomyelin have been studied using a combination of physical techniques. (ehu.es)
  • called arylsulfatase A (ASA), or cerebroside sulfatase . (britannica.com)
  • Very low arylsulfatase A and cerebroside sulfatase activities in leukocytes of healthy members of metachromatic leukodystrophy family. (thefreedictionary.com)
  • Cerebrosides is the common name for a group of glycosphingolipids called monoglycosylceramides which are important components in animal muscle and nerve cell membranes. (wikipedia.org)
  • The melting point of cerebrosides is considerably greater than physiological body temperature, >37.0 °C, giving glycolipids a paracrystalline, similar to liquid crystal structure. (wikipedia.org)
  • Due to the relatively high melting point of cerebrosides (much greater than physiological body temperature) they have a para-crystalline structure. (matreya.com)
  • cerebroside Any one of a class of glycolipids in which a single sugar unit is bound to a sphingolipid (see phospholipid ). (encyclopedia.com)
  • Cerebrosides are a kind of glycolipids highly enriched on the surface of myelin-producing cells and are composed by C18-4, 8-sphingadienine, α-hydroxy acid and a saccharide head. (sioc.ac.cn)
  • Cerebroside synthesis can therefore give a measurement of myelin formation or remyelination. (wikipedia.org)
  • Cerebrosides are found in the myelin sheath of nerve tissue, for example, kerasin, nervon, oxynervon, phrenosin, these names are also used for the fatty acid involved. (thefreedictionary.com)
  • cerebrosides are found in the myelin sheath of nerve tissue. (thefreedictionary.com)
  • Synthesis of a New Cerebroside from a Chondropsis sp. (nii.ac.jp)
  • S. Ogawa and H. Tsunoda , Chemical Synthesis of Glycosylamide and Cerebroside Analogs Composed of Carba Sugars. (elsevier.com)
  • In addition, there is reduction in accumulation of cholesterol, phospholipid, and cerebroside , which affects the cerebellar function. (thefreedictionary.com)
  • Isolation of Large Amounts of Cerebroside and Cholesterol Sulphate in the Sea Star Asterias rubens . (springer.com)
  • Increased cerebroside concentration in plasma and erythrocytes in Gaucher disease: significant differences between type I and type III. (thefreedictionary.com)
  • Cerebroside is sometimes prefixed by gluco-, galacto-, among others, in place of the correct glucosylceramide, and the like. (thefreedictionary.com)
  • The present study was undertaken to explore the preventive effect of dietary sea cucumber cerebroside (SCC) extracted from Acaudina molpadioides in fatty liver rats. (biomedcentral.com)
  • The deficiency of arylsulphatase A leads to storage of cerebroside sulphate in the neural tissues. (thefreedictionary.com)
  • Cerebroside antibody binding activity was only partially blocked by 0.5 M galactose or α- and β-methylgalactopyranoside, suggesting poor specificity of antiC for a specific glycosidic residue or linkage. (ajtmh.org)
  • SUMMARY: Water-insoluble complexes of ganglioside with cerebroside fixed tetanus toxin at low concentrations (a few LD 50/ml.) of toxin. (microbiologyresearch.org)
  • A complex containing 25% ganglioside with cerebroside was 50 times better at fixing toxin than complexes containing either 2% or 50% ganglioside. (microbiologyresearch.org)
  • The complex of cerebroside and ganglioside, containing 25% ganglioside, did not fix strychnine, serotonin, botulinum toxin or plasma albumin. (microbiologyresearch.org)
  • A lipid, such as a cerebroside, that contains carbohydrate groups. (dictionary.com)
  • Spathoside, a cerebroside and other antibacterial constituents of the stem bark of Spathodea campanulata. (thefreedictionary.com)
  • Antibacterial and xanthine oxidase inhibitory cerebrosides from Fusarium sp IFB-121, an endophytic fungus in Quercus variabilis. (thefreedictionary.com)
  • This leads to an accumulation of cerebroside and psychosine (which is very cytotoxic and can result in demyelination of nerves and loss of axonal conductivity). (matreya.com)
  • Rebuilding the balance of STAT-1 and STAT3 signalings by fusaruside, a cerebroside compound, for the treatment of T-cell-mediated fulminant hepatitis in mice. (thefreedictionary.com)
  • These hydrogen bonds within the cerebrosides result in the molecules having a high transition temperature and compact alignment. (wikipedia.org)
  • A natural cerebroside (antiC) IgM antibody was found at relatively high levels in the serum of every healthy individual studied. (ajtmh.org)
  • Eicosenoic acid is important in the production of cerebrosides , which are components of nerve membranes and the "white substance" of the brain. (thefreedictionary.com)
  • 7. Barreto-Bergter, E., Pinto, M.R. & Rodrigues, M.L. 2004 Structure and biological functions of fungal cerebrosides. (fgsc.net)
  • New cerebroside and chondrocyte proliferation activity of Caryota mitis L. (nih.gov)