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)
Hexoses are simple monosaccharides, specifically six-carbon sugars, which include glucose, fructose, and galactose, and play crucial roles in biological processes such as energy production and storage, and structural components of cells.
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.
Sulfatases are a group of enzymes that catalyze the hydrolysis of sulfate ester bonds in various substrates, playing crucial roles in the metabolism and homeostasis of carbohydrates, proteoglycans, neurotransmitters, and steroid hormones within the body.
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.

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)

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

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

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

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

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

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

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

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

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

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

Cerebroside-sulfatase is an enzyme that plays a crucial role in the breakdown and recycling of lipids within the body, particularly in the brain. Its primary function is to break down a type of lipid called cerebroside sulfate, which is a major component of the myelin sheath that surrounds and insulates nerve fibers in the brain and nervous system.

Cerebroside-sulfatase deficiency can lead to a group of genetic disorders known as the mucopolysaccharidoses (MPS), specifically MPS IIIB or Sanfilippo syndrome B. In this condition, the lack of cerebroside-sulfatase activity leads to an accumulation of cerebroside sulfate in the lysosomes of cells, resulting in progressive neurological deterioration and developmental delays.

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

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

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

Metachromatic leukodystrophy (MLD) is a genetic disorder that affects the nervous system's white matter. It is caused by mutations in the arylsulfatase A (ARSA) gene, which leads to an accumulation of sulfatides in the brain and peripheral nerves. This accumulation results in progressive damage to the protective sheath (myelin) that covers nerve fibers, impairing the transmission of nerve impulses and leading to neurological symptoms.

The clinical presentation of MLD varies depending on the age of onset. The late-infantile form is the most common and typically appears between ages 1 and 2. Symptoms include developmental regression, motor difficulties, muscle weakness, and loss of vision and hearing. The juvenile form usually begins between ages 4 and 6, while the adult form can manifest anytime after age 16. These later-onset forms tend to have a slower progression but still result in significant neurological impairment over time.

Currently, there is no cure for MLD, and treatment focuses on managing symptoms and slowing disease progression. Bone marrow transplantation or stem cell transplantation may be beneficial if performed early in the course of the disease.

I'm sorry for any confusion, but the term "Sea Cucumbers" is not typically used in medical definitions. It is a common name given to marine animals belonging to the class Holothuroidea in the phylum Echinodermata. These are sausage-shaped, bottom-dwelling creatures found on the sea floor worldwide. They have a leathery skin and a set of tube feet used for locomotion. While they have some cultural and commercial importance in parts of the world, they do not have direct relevance to medical definitions.

"Termitomyces" is a genus of fungi that have a mutualistic relationship with termites. These fungi grow inside the nests of certain termite species, particularly those in the family Macrotermitinae, and are cultivated by the termites for food. The termites feed on the fungus's hyphae and spores, while the fungi receive shelter and nutrients from the termites. This mutualistic relationship is one of the most complex examples of symbiosis in the animal kingdom.

The Termitomyces fungi produce large, fleshy mushrooms that grow above ground and are highly prized as a food source in some parts of the world, particularly in Africa. The mushrooms have a distinctive appearance, with caps that can range in size from a few centimeters to over 30 cm in diameter, and stems that can be several centimeters long.

It's worth noting that Termitomyces is not a medical term per se, but rather a taxonomic category used in the field of mycology (the study of fungi).

Galactosylceramides are a type of glycosphingolipids, which are lipid molecules that contain a sugar (glyco-) attached to a ceramide. Galactosylceramides have a galactose molecule attached to the ceramide. They are important components of cell membranes and play a role in cell recognition and signaling. In particular, they are abundant in the myelin sheath, which is the protective covering around nerve fibers in the brain and spinal cord. Abnormal accumulation of galactosylceramides can lead to certain genetic disorders, such as Krabbe disease and Gaucher disease.

N-Acylsphingosine Galactosyltransferase is a type of enzyme that plays a role in the synthesis of galactosylceramide, which is a critical component of the myelin sheath in the nervous system. The enzyme's systematic name is UDP-galactose:N-acylsphingosine galactosyltransferase, and it catalyzes the following chemical reaction:
UDP-galactose + N-acylsphingosine = UDP + D-galactosyl-N-acylsphingosine.
This enzyme is also known as galactosylceramide synthase, and it is involved in the biosynthesis of galactolipids, which are essential for the formation and maintenance of the myelin sheath around neurons. Deficiencies in this enzyme have been linked to certain genetic disorders, such as Krabbe disease and hereditary sensory and autonomic neuropathy type I.

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

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

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

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

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

"Schizophyllum" is not a term that has a medical definition on its own. However, it is the name of a genus of fungi that are commonly found in temperate and tropical regions worldwide. The most common and well-known species in this genus is Schizophyllum commune, which is known to cause a rare and mild form of respiratory infection in humans called pulmonary schizophyllosis.

Pulmonary schizophyllosis is caused by inhaling the spores of S. commune, which can lead to allergic reactions or, more rarely, invasive fungal infections in people with weakened immune systems. Symptoms of this condition may include coughing, chest pain, fever, and difficulty breathing.

It's worth noting that pulmonary schizophyllosis is a very rare disease, and most people who come into contact with S. commune fungi do not develop any symptoms or health problems. Nonetheless, it is important for medical professionals to be aware of this potential infection source in immunocompromised patients who present with respiratory symptoms.

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

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

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

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

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

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

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

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

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

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

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

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

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

The Harderian gland is a specialized exocrine gland located in many vertebrate species, including birds and mammals. In humans, it is rudimentary and not fully developed. However, in other animals like rodents, lagomorphs (rabbits and hares), and some reptiles, this gland plays a significant role.

The Harderian gland is primarily responsible for producing and secreting lipids, which help to lubricate the eye's surface and the nictitating membrane (third eyelid). This lubrication ensures that the eyes remain moist and protected from dryness and external irritants. Additionally, the secretions of the Harderian gland contain immunoglobulins, which contribute to the animal's immune defense system by providing protection against pathogens.

In some animals, the Harderian gland also has a role in pheromone production and communication. The study and understanding of this gland are particularly important in toxicological research, as it is often used as an indicator of environmental pollutant exposure and their effects on wildlife.

Gaucher disease is an inherited metabolic disorder caused by the deficiency of the enzyme glucocerebrosidase. This enzyme is responsible for breaking down a complex fatty substance called glucocerebroside, found in the cells of various tissues throughout the body. When the enzyme is not present in sufficient quantities or is entirely absent, glucocerebroside accumulates inside the lysosomes (cellular organelles responsible for waste material breakdown) of certain cell types, particularly within white blood cells called macrophages. This buildup of lipids leads to the formation of characteristic lipid-laden cells known as Gaucher cells.

There are three main types of Gaucher disease, classified based on the absence or presence and severity of neurological symptoms:

1. Type 1 (non-neuronopathic) - This is the most common form of Gaucher disease, accounting for approximately 95% of cases. It primarily affects the spleen, liver, and bone marrow but does not typically involve the central nervous system. Symptoms may include an enlarged spleen and/or liver, low red blood cell counts (anemia), low platelet counts (thrombocytopenia), bone pain and fractures, and fatigue.
2. Type 2 (acute neuronopathic) - This rare and severe form of Gaucher disease affects both visceral organs and the central nervous system. Symptoms usually appear within the first six months of life and progress rapidly, often leading to death before two years of age due to neurological complications.
3. Type 3 (subacute neuronopathic) - This form of Gaucher disease affects both visceral organs and the central nervous system but has a slower progression compared to type 2. Symptoms may include those seen in type 1, as well as neurological issues such as seizures, eye movement abnormalities, and cognitive decline.

Gaucher disease is inherited in an autosomal recessive manner, meaning that an individual must inherit two defective copies of the gene (one from each parent) to develop the condition. Treatment options for Gaucher disease include enzyme replacement therapy (ERT), substrate reduction therapy (SRT), and chaperone therapy, depending on the type and severity of the disease.

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

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

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

Hexoses are simple sugars (monosaccharides) that contain six carbon atoms. The most common hexoses include glucose, fructose, and galactose. These sugars play important roles in various biological processes, such as serving as energy sources or forming complex carbohydrates like starch and cellulose. Hexoses are essential for the structure and function of living organisms, including humans.

The myelin sheath is a multilayered, fatty substance that surrounds and insulates many nerve fibers in the nervous system. It is essential for the rapid transmission of electrical signals, or nerve impulses, along these nerve fibers, allowing for efficient communication between different parts of the body. The myelin sheath is produced by specialized cells called oligodendrocytes in the central nervous system (CNS) and Schwann cells in the peripheral nervous system (PNS). Damage to the myelin sheath, as seen in conditions like multiple sclerosis, can significantly impair nerve function and result in various neurological symptoms.

Saposins are a group of naturally occurring lipid-binding proteins that play an essential role in the metabolism of lipids within cells. They are named after a skin disease called "Niemann-Pick disease," where defects in saposin function lead to an accumulation of lipids in various tissues, including the brain.

There are four types of saposins (SapA, SapB, SapC, and SapD) that are produced by the cleavage of a larger precursor protein called prosaposin. These proteins help to facilitate the breakdown of lipids in lysosomes, which are specialized organelles within cells that break down and recycle various materials.

Saposins play an important role in activating certain enzymes that are involved in breaking down lipids, such as sphingolipids and gangliosides. They do this by binding to these enzymes and presenting them with their lipid substrates in a way that allows the enzymes to efficiently break them down.

Defects in saposin function can lead to a variety of diseases, including Niemann-Pick disease, Gaucher disease, and Krabbe disease, which are characterized by an accumulation of lipids in various tissues and neurological symptoms.

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

The two main types of fatty acids are:

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

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

"Asterias" is a genus of starfish belonging to the family Asteriidae. The name "Asterias" itself does not have a specific medical definition, as it is a term used in the field of marine biology. However, some species of starfish in this genus may have potential medicinal or toxicological significance. For example, the venom of some asteriid starfish has been studied for its potential therapeutic applications, while others may contain toxins that can cause harm if ingested or come into contact with human skin.

It's worth noting that while some species of starfish may have medical relevance, the term "Asterias" does not have a direct medical definition and is not commonly used in medical contexts.

Sulfatases are a group of enzymes that play a crucial role in the metabolism of sulfated steroids, glycosaminoglycans (GAGs), and other sulfated molecules. These enzymes catalyze the hydrolysis of sulfate groups from these substrates, converting them into their respective unsulfated forms.

The human genome encodes for several different sulfatases, each with specificity towards particular types of sulfated substrates. For instance, some sulfatases are responsible for removing sulfate groups from steroid hormones and neurotransmitters, while others target GAGs like heparan sulfate, dermatan sulfate, and keratan sulfate.

Defects in sulfatase enzymes can lead to various genetic disorders, such as multiple sulfatase deficiency (MSD), X-linked ichthyosis, and mucopolysaccharidosis (MPS) type IIIC (Sanfilippo syndrome type C). These conditions are characterized by the accumulation of sulfated molecules in different tissues, resulting in progressive damage to multiple organs and systems.

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

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

Amino alcohols are organic compounds containing both amine and hydroxyl (alcohol) functional groups. They have the general structure R-NH-OH, where R represents a carbon-containing group. Amino alcohols can be primary, secondary, or tertiary, depending on the number of alkyl or aryl groups attached to the nitrogen atom.

These compounds are important in many chemical and biological processes. For example, some amino alcohols serve as intermediates in the synthesis of pharmaceuticals, dyes, and polymers. In biochemistry, certain amino alcohols function as neurotransmitters or components of lipids.

Some common examples of amino alcohols include:

* Ethanolamine (monoethanolamine, MEA): a primary amino alcohol used in the production of detergents, emulsifiers, and pharmaceuticals
* Serinol: a primary amino alcohol that occurs naturally in some foods and is used as a flavoring agent
* Choline: a quaternary ammonium compound with a hydroxyl group, essential for human nutrition and found in various foods such as eggs, liver, and peanuts
* Trimethylamine (TMA): a tertiary amino alcohol that occurs naturally in some marine animals and is responsible for the "fishy" odor of their flesh.

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

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

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

Hydroxy acids are a class of chemical compounds that contain both a carboxylic acid group and a hydroxyl group. They are commonly used in dermatology and cosmetic products for their exfoliating, moisturizing, and anti-aging properties. The two main types of hydroxy acids used in skincare are alpha-hydroxy acids (AHAs) and beta-hydroxy acids (BHAs).

Alpha-hydroxy acids include compounds such as glycolic acid, lactic acid, malic acid, tartaric acid, and citric acid. They work by breaking down the "glue" that holds dead skin cells together, promoting cell turnover and helping to improve the texture and tone of the skin. AHAs are also known for their ability to improve the appearance of fine lines, wrinkles, and age spots.

Beta-hydroxy acids, on the other hand, are primarily represented by salicylic acid. BHAs are oil-soluble, which allows them to penetrate deeper into the pores and exfoliate dead skin cells and excess sebum that can lead to clogged pores and acne breakouts.

It is important to note that hydroxy acids can cause skin irritation and sensitivity to sunlight, so it is recommended to use sunscreen and start with lower concentrations when first incorporating them into a skincare routine.

Insensible water loss is the unnoticeable or unperceived loss of water from the body through processes such as respiration, evaporation from the skin, and perspiration that is too fine to be seen or felt. It is a normal physiological process and typically accounts for about 400-800 milliliters (ml) of water loss per day in a healthy adult at rest. However, this amount can increase with factors such as environmental temperature, humidity, and altitude, as well as physical activity or illness that increases metabolic rate or alters body temperature regulation.

Insensible water loss is an important factor to consider in maintaining fluid balance in the body, particularly in individuals who are unable to regulate their own fluid intake, such as critically ill patients or those with impaired consciousness. Prolonged or excessive insensible water loss can lead to dehydration and electrolyte imbalances, which can have serious consequences on various organ systems and overall health.

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

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

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

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

Sulfotransferases (STs) are a group of enzymes that play a crucial role in the process of sulfoconjugation, which is the transfer of a sulfo group (-SO3H) from a donor molecule to an acceptor molecule. These enzymes are widely distributed in nature and are found in various organisms, including humans.

In humans, STs are involved in the metabolism and detoxification of numerous xenobiotics, such as drugs, food additives, and environmental pollutants, as well as endogenous compounds, such as hormones, neurotransmitters, and lipids. The sulfoconjugation reaction catalyzed by STs can increase the water solubility of these compounds, facilitating their excretion from the body.

STs can be classified into several families based on their sequence similarity and cofactor specificity. The largest family of STs is the cytosolic sulfotransferases, which use 3'-phosphoadenosine 5'-phosphosulfate (PAPS) as a cofactor to transfer the sulfo group to various acceptor molecules, including phenols, alcohols, amines, and steroids.

Abnormalities in ST activity have been implicated in several diseases, such as cancer, cardiovascular disease, and neurological disorders. Therefore, understanding the function and regulation of STs is essential for developing new therapeutic strategies to treat these conditions.

I believe there might be a slight confusion in your question. Sulfuric acid is not a medical term, but instead a chemical compound with the formula H2SO4. It's one of the most important industrial chemicals, being a strong mineral acid with numerous applications.

If you are asking for a definition related to human health or medicine, I can tell you that sulfuric acid has no physiological role in humans. Exposure to sulfuric acid can cause irritation and burns to the skin, eyes, and respiratory tract. Prolonged exposure may lead to more severe health issues. However, it is not a term typically used in medical diagnoses or treatments.

"Sterculia" is a botanical term that refers to a genus of trees in the family Sterculiaceae. The name "Sterculia" comes from the Latin word "stercus," which means excrement, due to the unpleasant smell of the tree's flowers. Several species of Sterculia have been used in traditional medicine for various purposes, including as a laxative and as a treatment for skin conditions. However, it is important to note that the use of Sterculia as a medical treatment has not been extensively studied and its safety and efficacy are not well-established. Therefore, it should not be used as a substitute for proven medical therapies.

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

Yanagihara, T; Cumings, JN (1969). "Fatty acid composition of cerebrosides and cerebroside sulphatides in cerebral oedema". ... 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 ...
... (EC 3.1.6.8, arylsulfatase A, cerebroside sulfate sulfatase) is an enzyme with systematic name ... Cerebroside-sulfatase at the U.S. National Library of Medicine Medical Subject Headings (MeSH) Portal: Biology (EC 3.1.6). ... 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. Arylsulfatase A Mehl E, ...
Configuration of cerebrosides. J. Biol. Chem. 221: 879-884. Carter, H. E., C. P. Schaffner, and D. Gottlieb. 1954. Levomycin. I ... Structure of the cerebrosides. J. Biol. Chem. 199: 283-288. Carter, H. E., D. Gottlieb, and H. W. Anderson. 1948. Chloromycetin ...
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. ...
MLD involves cerebroside sulfate accumulation. Metachromatic leukodystrophy, like most enzyme deficiencies, has an autosomal ...
1. Imaging of Cerebrosides Directly from Rat Brain Tissue". Analytical Chemistry. 79 (6): 2373-85. doi:10.1021/ac062251h. PMID ...
Cerebrosides: a group glycosphingolipids involved in nerve cell membranes. Galactocerebrosides: a type of cerebroseide with ... Jurevics H, Hostettler J, Muse ED, Sammond DW, Matsushima GK, Toews AD, Morell P (May 2001). "Cerebroside synthesis as a ... galactose as the saccharide moiety Glucocerebrosides: a type of cerebroside with glucose as the saccharide moiety; often found ...
He found that cerebroside accumulated in Gaucher's disease. In 1935 Klenk discovered a new group of glycosphingolipids in the ... Die Chemie der Cerebroside und Phosphatide. Springer. 9 March 2013. ISBN 9783642906626. Klenk, Grete (1966). "Biography of ... doi:10.1016/0079-6832(63)90018-1. Thierfelder, Hans; Klenk, Ernst (1930). Die Chemie der Cerebroside und Phosphatide. [With a ... Klenk did research on phospholipids, glycolipids, cerebrosides, glucocerebrosides, sphingosines, sphingolipids, ...
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 ...
Metz RJ, Radin NS (November 1982). "Purification and properties of a cerebroside transfer protein". The Journal of Biological ...
Specifically, cerebroside sulfotransferase (CST) is elevated as it passes along a signaling pathway which involves: Hepatocyte ... This reaction is catalyzed by cerebroside sulfotransferase (CST). CST is a homodimeric protein that is found in the Golgi ... In peripheral nerves that are cerebroside sulfotransferase (CST) deficient, the nodes of Ranvier form enlarged axonal ... Consequently, the COS-7 cells were transfected with galactosyltransferase and cerebroside sulfotransferase genes from the Madin ...
... s G and H, additional cerebrosides from the edible Chinese mushroom Termitomyces albuminosus v t e (Glycolipids ... Termitomycesphins A-H are neuritogenic cerebrosides isolated from the mushroom Termitomyces albuminosus. ...
Fujino Y, Nakano M (1969). "Enzymic synthesis of cerebroside from ceramide and uridine diphosphate galactose". Biochem. J. 113 ...
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. ...
Examples of these are the simple and complex glycosphingolipids such as cerebrosides and gangliosides. Sterols, such as ... cerebroside) and sphingolipids (sphingomyelin). The terms lipoid, lipin, lipide and lipid have been used with varied meanings ...
Plasma membranes also contain carbohydrates, predominantly glycoproteins, but with some glycolipids (cerebrosides and ...
It is found in wood tar, various cerebrosides, and in small amounts in most natural fats. The fatty acids of peanut oil contain ...
Birds and bats also share a high concentration of cerebrosides in the skin of their wings. This improves skin flexibility, a ...
1991). "The organization of the gene for the human cerebroside sulfate activator protein". FEBS Lett. 280 (2): 267-70. doi: ... 1990). "The complete amino-acid sequences of human ganglioside GM2 activator protein and cerebroside sulfate activator protein ...
... (also called glucosylceramide) is any of the cerebrosides in which the monosaccharide head group is glucose. ...
... cerebrosides and globosides. Epidermal ceramides are critical for normal skin barrier function and SPTLC2 is differentially ...
Morell P, Radin NS (1969). "Synthesis of cerebroside by brain from uridine diphosphate galactose and ceramide containing ...
The simplest glycolipid is cerebroside, in which there is only one sugar residue, either Glc or Gal. More complex glycolipids, ...
1990). "The complete amino-acid sequences of human ganglioside GM2 activator protein and cerebroside sulfate activator protein ...
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 ...
... (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 ...
In 1900 he worked with Wörner on brain chemicals and identified cerebron or phrensosin which is now known as a cerebroside. He ...
Other names in common use include GSase, 3'-phosphoadenosine-5'-phosphosulfate-cerebroside sulfotransferase, galactocerebroside ...
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 ...
Yanagihara, T; Cumings, JN (1969). "Fatty acid composition of cerebrosides and cerebroside sulphatides in cerebral oedema". ... 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 ...
LOINC Group Code LG9700-8 Cerebroside sulfatase activator,Imp,Pt,Fibroblasts ... Cerebroside sulfatase activator [Interpretation] in Fibroblast. 49321-3. Cerebroside sulfatase activator [Interpretation] in ... Cerebroside sulfatase activator,Imp,Pt,Fibroblasts Active Maturity: Beta *The LOINC Groups project is a work in progress. The ...
Deuterium Nuclear Magnetic Resonance Studies of N-Palmitoylglucosy leer amide (Cerebroside) Head Group Structure. Biochemistry ... Deuterium Nuclear Magnetic Resonance Studies of N-Palmitoylglucosy leer amide (Cerebroside) Head Group Structure, Biochemistry ... Deuterium Nuclear Magnetic Resonance Studies of N-Palmitoylglucosy leer amide (Cerebroside) Head Group Structure. Biochemistry ... Deuterium Nuclear Magnetic Resonance Studies of N-Palmitoylglucosy leer amide (Cerebroside) Head Group Structure. In: ...
The content of myelin-enriched cerebrosides, sulfatides, and plasmalogen ethanolamines was significantly lower in optic nerve ...
Glucosyl cerebroside lipidosis. *Glucosylceramidase deficiency. *Glucosylceramide beta-glucosidase deficiency. * ...
enables cerebroside transfer activity IDA Inferred from Direct Assay. more info. PubMed ...
Cerebrosides in Mature-Green and Red-Ripe Bell Pepper and Tomato Fruits Bruce D. Whitaker ...
cerebrosides. *gangliosides Discussion. Links. AB Isogenes RecentChanges Link Phylogenetics Trail: Glycosphingolipid. This page ...
Xin, H.L.; Hou, Y.H.; Xu, Y.F.; Yue, X.Q.; Li, M.; Lu, J.C.; Ling, C.Q. Portulacerebroside A: New cerebroside from Portulaca ... Ling, T.J.; Xia, T.; Wan, X.C.; Li, D.X.; Wei, X.Y. Cerebrosides from the Roots of Serratula chinensis. Molecules 2006, 11, 677 ... The 1H- and 13C-NMR spectra (Table 1 and Table 2) showed characteristics of a cerebroside with a 2-hydroxy fatty acid fraction ... Yang, N.Y.; Ren, D.C.; Duan, J.A.; Xu, X.H.; Xie, N.; Tian, L.J. Ceramides and cerebrosides from Ligusticum chuanxiong Hort. ...
cerebroside +. 23. ganglioside +. 12. glycosylceramide +. 23. oligoglycosylceramide +. 3. omega-hydroxy-ultra-long chain fatty ...
Sphingolipids include ceramides, sphingomyelins, cerebrosides, sulfatides, globosides and gangliosides (Figure 1). De novo ... cerebrosides, globosides, gangliosides). In addition, ceramide can be converted to sulfatides by the action of ... galactosylceramide synthase followed by cerebroside sulfotransferase (CST). Additionally, ceramide is also converted to ...
Cerebrosides are found in the brain and in the myelin sheath of nerve tissue. The structure of the cerebroside phrenosine. (a) ... Cerebrosides are found in the brain and in the myelin sheath of nerve tissue. The structure of the cerebroside phrenosine. (a) ...
Keywords: hydrolase, cerebroside-3-sulfate hydrolysis, lysosomal enzyme, formylglycine. Deposited on 2000-06-13, released 2001- ...
Furthermore, other bioactive substances, including triterpenes, proteins, lipids, cerebrosides, and phenols, have been ...
Cerebroside galactosidase: a method for determination and a comparison with other lysosomal enzymes in developing rat brain. J ...
... sphingomyelin and cerebroside; made up of 75% lipid and 15% protein ...
Proactivator polypeptide; Contains: Saposin-A; Protein A; Contains: Saposin-B-Val; Contains: Saposin-B; Cerebroside sulfate ... Proactivator polypeptide; Contains: Saposin-A; Protein A; Contains: Saposin-B-Val; Contains: Saposin-B; Cerebroside sulfate ...
Cerebroside sulfatase deficiency. *Metachromatic leukoencephalopathy. *Sulfatide lipidosis. References. *Adang et al., 2017, ...
Proactivator polypeptide; Contains: Saposin-A; Protein A; Contains: Saposin-B-Val; Contains: Saposin-B; Cerebroside sulfate ...
Glucosyl Cerebroside Lipidosis. *Cerebroside Lipidoses, Glucosyl. *Cerebroside Lipidosis, Glucosyl. *Glucosyl Cerebroside ...
... cerebrosides. The observed phase coexistence scenario consists of elongated, micrometer-sized cerebroside-rich solid ordered ... The POPC/cerebroside mixture display solid ordered/liquid disordered phase coexistence in a broad range of compositions and ... We studied the thermal behavior of membranes composed of mixtures of natural cerebrosides (from porcine brain) and 1-palmitoyl- ... Direct visualization of the lateral structure of porcine brain cerebrosides/POPC mixtures in presence and absence of ...
... cerebrosides, and ceramide. J. Biol. Chem. 2001, 276, 33540-33546. [Google Scholar] [PubMed] ...
The berries contain a compound called cerebrosides ks and is better at protecting the liver than milk thistle. ...
Cerebroside galactosidase: a method for determination and a comparison with other lysosomal enzymes in developing rat brain. J ...
Industrialize justify little shoot-em-up paratrooper, nobody cerebrosides grubstaked hoe veel topiramate topiramaat amsterdam ... nobody cerebrosides grubstaked himself booted autohypnotic laterad and still amputate nondictatorially. Unpreferred cowpoke, ...
Cerebroside sulfate, an integral constituent of the myelin sheaths surrounding neuronal axons in the brain, helps to maintain ...
Cerebroside-Sulfatase Medicine & Life Sciences 100% * arylsulfatase Agriculture & Biology 80% * genotype Earth & Environmental ...
Sphingomyelin was increased in C-BS and cerebellum, but no changes were found for cerebroside (a myelin-enriched ...
Synonym: Gm2-Ap, Cerebroside Sulfate Activator Protein, Shi Activator Protein 3, Sap-3;. Engineered: Yes. Organism_scientific: ...
  • It is phosphorylated by ceramide kinase (CK) to form ceramide-1-phosphate or it can be glycosylated by glucosylceramide synthase to form glycosphingolipids (cerebrosides, globosides, gangliosides). (frontiersin.org)
  • Examples are cerebrosides and gangliosides. (brainwritings.com)
  • Saposin-B stimulates the hydrolysis of galacto-cerebroside sulfate by arylsulfatase A (EC 3.1.6.8), GM1 gangliosides by beta-galactosidase (EC 3.2.1.23) and globotriaosylceramide by alpha-galactosidase A (EC 3.2.1.22). (affbiotech.cn)
  • Synthesis of cerebrosides and gangliosides proceeds with great velocity in the developing brain during myelination. (doclecture.net)
  • In adults, almost all of cerebrosides (90%) are in the myelin sheath, and gangliosides are in neurons. (doclecture.net)
  • 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)
  • Cerebrosides are glycosphingolipids found in the cell membranes of the neurons and the muscle cells. (brainwritings.com)
  • In Krabbe disease, an enzyme needed to break down lipids, called cerebroside beta-galactosidase, is not working correctly. (msdmanuals.com)
  • Cerebroside synthesis can therefore give a measurement of myelin formation or remyelination. (wikipedia.org)
  • Cerebrosides containing a sulfuric ester (sulfate) group, known as sulfatides, also occur in the myelin sheath of nerves. (wikipedia.org)
  • Cerebrosides are found in the brain and in the myelin sheath of nerve tissue. (quizlet.com)
  • Cerebroside sulfate, an integral constituent of the myelin sheaths surrounding neuronal axons in the brain, helps to maintain optimal neurological health. (holisticprimarycare.net)
  • In addition, ceramide can be converted to sulfatides by the action of galactosylceramide synthase followed by cerebroside sulfotransferase (CST). (frontiersin.org)
  • Site‐Selective trans‐Hydrostannation of 1,3‐ and 1,n‐Diynes: Application to the Total Synthesis of Typhonosides E and F, and a Fluorinated Cerebroside Analogue. (mpg.de)
  • 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)
  • The cerebroside has a sphingosine core. (brainwritings.com)
  • Cerebroside E, a compound found in the fruiting bodies of Hericium erinaceus, was shown to be particularly beneficial in cancer treatment. (forij.co)
  • The fundamental structure of a cerebroside is ceramide. (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)
  • We also show that the order parameter describing the head group wobble is fully determined for each system, and it indicates a rather mobile structure for the cerebroside head group, in a variety of environments. (illinois.edu)
  • The structure of the cerebroside phrenosine. (quizlet.com)
  • Monoglycosyl and oligoglycosylceramides having a mono or polysaccharide bonded glycosidically to the terminal OH group of ceramide are defined as cerebrosides. (wikipedia.org)
  • Cerebrosides which contain as their polar head group a galactose moiety bound in glycosidic linkage to the hydroxyl group of ceramide. (uchicago.edu)
  • A 2015 study concluded that cerebroside E inhibited angiogenesis (the formation of new blood cells) in cancer cells and promoted cancer apoptosis. (forij.co)
  • Other common groups bonded to the terminal oxygen atom include phosphocholine , yielding a sphingomyelin , and various sugar monomers or dimers, yielding cerebrosides and globosides , respectively. (cloudfront.net)
  • Sphingolipids from this study included ceramides (Cer), cerebrosides (GlcCer), and sphingomyelin (SM). (vanderbilt.edu)
  • Cerebrosides and gangliosides are examples of sphingolipids, and they are commonly found in nerve cells and brain tissues, helping with proper nerve function. (themasterchemistry.com)
  • Two common classes of glycosphingolipids are gangliosides and cerebrosides. (tangut.net)
  • 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)
  • 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 containing a sulfuric ester (sulfate) group, known as sulfatides, also occur in the myelin sheath of nerves. (wikipedia.org)
  • Cerebrosides have a single glucose or galactose at the 1-hydroxy position. (cloudfront.net)
  • Galactose is a crucial component of cerebroside, which can aid in an infant's brain growth. (farmdidi.com)
  • With antioxidant and anticancer allows the strengthening of the immune system, conferring protection against diseas¬ es of the cardiovascular system, nervous system (cerebrosides) of vision (carotene and zeaxanthin), liver and kidney. (produnats.pt)
  • Arylsulphatase A (ASA, EC 3.1.6.1) is a lysosomal enzyme that catalyses cerebroside sulphate degradation. (bmj.com)
  • ASA deficiency is associated with metachromatic leucodystrophy (MLD), a rare autosomal recessive disorder, which is characterised by the storage of cerebroside sulphate. (bmj.com)
  • In Krabbe disease, an enzyme needed to break down lipids, called cerebroside beta-galactosidase, is not working correctly. (msdmanuals.com)
  • 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)
  • These hydrogen bonds within the cerebrosides result in the molecules having a high transition temperature and compact alignment. (wikipedia.org)
  • Cerebrosides, on the other hand, are abundant in the brain's white matter and other nervous tissues. (tangut.net)
  • 11. Cerebrosides with antiproliferative activity from Euphorbia peplis L. (nih.gov)