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At least 721 records · Page 40Linked to original sources
PubMed1978

A histochemical study in Huntington's disease and control cases.

1. Extracellular deposits of cerebrosides and free fatty acids were found in the formaldehyde fixed frozen sections of the frontal lobe in 8 cases of Huntington's disease, in one case of the infantile form of Gaucher's disease, 2 cases of Krabbe's globoid cell leucodystrophy, 2 cases of metachromatic leucodystrophy, one case with multiple sclerosis, 2 cases with cerebral contusion and one case with bacterial meningitis. 2. The cerebroside deposits were present in the white matter as well as in the grey matter. 3. The significance of these findings in relation to their etiology is discussed.

Adolescent↗
PubMed1992

Neutral glycolipids of human and bovine milk.

The neutral glycolipids of milk, a small fraction of the total lipids, are of potential biological importance. The simultaneous quantitation of the simple (less than five sugars) glycosphingolipids of human milk samples was achieved by high-pressure liquid chromatography. The samples, representing various stages of lactation, parity of the nursing child, and age of the mother, contained similar glycolipid patterns, but with varying individual glycolipid concentrations. The cerebrosides are major glycosphingolipids of human milk: the non-hydroxylated fatty acid (NFA)-containing species are present at 1.8 microM, and the hydroxylated and/or short-chain fatty acid-containing species (HFA) are present at 1.7 microM; NFA lactosylceramide is present at 931 nM. The cerebrosides appear to be primarily galactosylceramides (galactocerebrosides); glucosylceramides (glucocerebrosides) are a minor component. Globotriaosylceramide (Gb3) is found at 50 nM and 73 nM for the NFA and HFA species, respectively, while globoside (Gb4) is found at 45 nM and 46 nM for the NFA and HFA species. Bovine milk glycosphingolipids differ from those of human milk, with bovine milk containing mainly NFA glucosylceramide (8 microM) and NFA lactosylceramide (17 microM); bovine milk contains little Gb3 or Gb4.

Animals↗
PubMed1977

Lysosomal non-lipid component of Gaucher's cells.

An ultrastructural, histochemical and chemical analysis of storage elements in the infantile form of Gaucher's disease showed that in addition to cerebroside the lysosomes also included a non-lipid component of protein, or possibly glycoprotein nature. This component, easily removable with trypsin, was present in such quantities that it conditioned the typical solid and fibrillar appearance of storage elements even after they had been substantially delipidized. Another noteworthy finding was that the ultrastructural appearance of tubular structures generally regarded as stored cerebrosides persisted in all the extracted specimens without any noticeable change. The findings are compared with available data from the literature and their significance briefly discussed.

Cerebrosides↗
PubMed1980

Lipid domain formation and ligand-induced lymphocyte membrane changes.

Spectral parameters of spin-labelled phosphatidylcholine, ceramide and cerebroside in the plasma membranes of human blood lymphocytes were measured before and after treatment with various ligands, which included concanavalin-A and phytohemagglutinin. It was found that ligand treatment led to a significant decrease in order of the hydrocarbon chains of the phospholipids. This was accompanied by a clustering of the labelled spingolipids, as estimated by spin-spin interaction, and an increase in the order of their hydrocarbon chains. In the untreated cells the cerebroside fatty acid chain was more ordered than that of the phosphatidylcholine. It was considered that the decrease in phospholipid order was brought about by the sequestration of the more rigid sphingolipids into the patches and caps formed by receptor-ligand complexes. The significance of these changes in lipid distribution and ordering is discussed in relation to the activation of membrane enzyme systems by mitogenic ligands.

Cell Membrane↗
PubMed1986

Circular dichroism spectra of aqueous dispersions of sphingolipids.

The circular dichroism (CD) spectra of a number of sphingolipids dispersed in water have been studied. The lipids include cerebrosides such as palmitoyl cerebroside, glucocerebroside from the spleen of Gaucher patients, bovine brain galactocerebrosides type I and type II, (BCI and BCII, respectively) and also sphingomyelins such as egg sphingomyelin and bovine brain sphingomyelin. Changes in the CD spectra of the lipids which occur upon heating and cooling and the effects of cholesterol, phosphatidylcholine and the opiate leucine enkephalin were studied.

Animals↗
PubMed1986

Spontaneous vesicularization of myelin lipids is counteracted by myelin basic protein.

Hand-vortexed dispersions of several lipids (cerebrosides, sulfatides, PC, PE, PS and sphingomyelin), mixed in the ratios found for these categories of lipids in myelin, exhibit 31P-NMR spectra which have contributions from both isotropic and lamellar resonances. Investigation of this system by freeze-fracture electron microscopy and X-ray diffraction revealed that this lipid mixture has spontaneously formed small unilamellar vesicles (SUVs) (diam. approximately 400 A) and large highly convoluted unilamellar vesicles (LUVs) (diam. approximately 1000 A), the latter possibly resulting from aggregation and fusion of the SUV structures. This vesicularization of the myelin lipids was reversed by the addition of myelin basic protein: only large multilamellar aggregates were formed in the presence of protein, as shown by all three experimental methods. Although no rigorous physical-chemical explanation for these phenomena is yet available, the possibility is suggested that the high concentration of cerebrosides and/or phosphatidylethanolamine in this particular mixture of myelin lipids play pivotal roles in the formation of these unusual vesicles. Spontaneous vesicularization of myelin lipids is discussed as a potential pathway toward destabilization of the myelin sheath.

Centrifugation, Density Gradient↗
PubMed1986

Effect of ethylene glycol on the phase transition kinetics of gluco- and galactocerebrosides.

The effect of different concentrations of ethylene glycol in water on the phase transition (metastable----stable state) of Gaucher's glucocerebroside, of bovine brain cerebroside type II (non hydroxy acyl chains only) and of N-palmitoylgalactocerebroside has been investigated. The phase transition and its kinetics were inferred from the thermograms at different heating and cooling rates and confirmed by FTIR spectra of the cerebrosides in the different states. The significance of the conformational differences of the glucose and of the galactose residues with respect to their solvation, and the subsequent effect on the intermolecular interactions and the phase transition is discussed.

Calorimetry, Differential Scanning↗
PubMed1976

Microheterogeneity of arylsulfatase A from human tissues.

Human arylsulfatase A (cerebroside-3-sulfate 3-sulfohydrolase, EC 3.1.6.8) exhibited microheterogeneity on isoelectric focusing in polyacrylamide gels. Pure urinary enzyme gave 3 bands of activity with pI values of 4.7, 4.8 and 4.9, whereas purified liver enzyme yielded six equally spaced bands from pI 4.4 to 4.9. Detection of enzyme in the gel was made by either methylumbelliferyl sulfate or nitrocatechol sulfate. Crude enzyme preparations from human liver, kindey, placenta, brain and testis showed the six-banded pattern with varying amounts of activity in the different bands. The banding pattern of cultured human fibroblast extracts was distinctive: in addition to activity in the area of Bands 1-6 a sharp band at pI 5.1 was observed with both enzyme stains. This latter band was also present in metachromatic leukodystrophy fibroblast extracts. However, in this case the band did not appear when the specific aryl-sulfatase A stain was used. Enzyme Bands 1, 2 and 3 from urine were isolated by extraction of the gel. The three bands refocused in their initial positions; showed nearly identical enzymatic activities toward methylumbelliferyl sulfate, mitrocatechol sulfate, cerebroside sulfate and ascorbic acid 2-sulfate; and demonstrated equivalent immunological competence by antibody titration. The banding pattern of urinary arylsulfatase A was unchanged with neuraminidase treatment, whereas Bands 4-6 of the liver enzyme were converted to Bands 1-3 by this treatment. It appears that Bands 4-6 are due to sialylation of aryl-sulfatase A but that Bands 1-3 are probably due to some other type of post-ribosomal protein modification.

Brain↗
PubMed1975

3-Ketosphingolipids: application to the determination of sphingolipids which contain 4-sphingenine.

1. Ceramides and cerebrosides, both with 2-hydroxy or non-hydroxy fatty acids, and sphingomyelins were converted quantitatively to the corresponding 3-keto derivatives with the oxidative reagent 2,3-dichloro-5,6-dicyanobenzoquinone. Sulfatides were converted to 3-ketocerebrosides. 2. The conversion of these sphingolipids to the alpha,beta-unsaturated ketone permits three methods of quantitation: in Method I, the absorption of the reaction product is measured at 230 nm; Method II utilizes high performance liquid chromatography and an ultraviolet light detector; in Method III, the product is reduced with NaB3H4 and the radioactivity of the reduced products is determined. All three methods can be used to measure sphingolipids in nanomole quantities. 3. Methods I and II have been applied to the determination of cerebroside and sulfatide content of normal and metachromatic leukodystrophy brains. In the white matter of the pathological brains, there was a greater accumulation of sulfatides containing non-hydroxy fatty acids than of those containing 2-hydroxy fatty acids. The ceramide levels of the cerebellum in a Farber's disease patient and a control were determined by Method II. Method III was used to determine the content of free ceramides in human sera.

Brain↗
PubMed1982

Determination of glucocerebroside, sphingomyelin, free fatty acid and total lipid by thin-layer chromatography and charring-scintillation quenching.

A previously described method has been extended to various specific lipids of liver and brain. The basic method involves thin-layer chromatography followed by charring to reveal the bands. The intensity of each band is determined by suspending the silica gel in a radioactive scintillation gel and measuring the optically quenched activities. The lipids are extracted with hexane/isopropanol and, in the case of total lipid determinations, the extract is simply applied to a silica gel plate and charred without use of a development step. For brain cerebroside, the extract is applied to the plate and developed in the usual way. For liver cerebroside, the dried lipid extract is fractionated with a silica gel column to purify the glycolipid, which is then purified further by development with a plate. For sphingomyelin the ester type lipids in the extract are cleaved by alkali for 1 min and the resultant lipids are applied directly to the thin-layer plate. Free fatty acids are chromatographed and measured after a preliminary solvent partitioning to remove most lipids. The method is useful for samples of 5-40 micrograms. Methods for quantitative application of samples to plates are described. A modification of the Camag sample streaker is described which yields precise 1-cm streaks.

Animals↗
PubMed1983

An improved procedure for the quantitative determination and characterization of sulfatides in rat kidney and brain by high-performance liquid chromatography.

A significant improvement has been made in the desulfation step of our previously published HPLC determination of cerebrosides, sulfatides, and monogalactosyl diacylglycerols (Nonaka, G. and Kishimoto, Y., Biochim. Biophys. Acta 572 (1978) 423-431). Instead of the original two-phase reaction, a solution of trifluoroacetic acid in ethyl acetate is used for the solvolysis in the new method. The revised method was used to determine the levels of cerebrosides and sulfatides in rat kidney. Among four individual glycosphingolipids studied, hydroxysulfatide was present at the highest level (0.7-1.3 nmol/mg of dry tissue), followed by nonhydroxysulfatide (0.3-0.8 nmol/mg of dry tissue). Hydroxycerebroside (0.09-0.16 nmol/mg of dry tissue) and nonhydroxycerebroside (0.03-0.09 nmol/mg of dry tissue) were present in smaller quantities. There appear to be no significant differences between male and female animals of different ages (30-120 days), although the amounts decreased slightly in older animals and there was a higher concentration in female than in male kidney. Tissue size was significantly smaller in females. The homolog composition of rat kidney sulfatide was studied by reverse-phase HPLC, and was found to be significantly different from that reported in human kidney. Rat sulfatides contained fatty acids with a higher degree of saturation and longer chain length. Preliminary studies indicated that rat kidney contained unusually large quantities of C25:1 and C27:1 fatty acids and also that there was more C26:1 than C24:1 acid. In brain of the same animals the ratio of nonhydroxy to hydroxysulfatide decreased with age (1.5:1 in 30-day-old brain; 1:1 at 90 days).

Aging↗
PubMed1992

Membrane lipids of human peripheral nerve and spinal cord.

Major membrane lipids were determined in specimens of human peripheral nerve (cauda equina) and spinal cord of 10 subjects aged 20-70 years. The same lipids were also assayed in myelin from the same tissues isolated with two different procedures and in myelin of cauda equina from 3 subjects aged 17-91 years isolated with a third method. The concentrations (mean and standard deviation) of phospholipids were 90 +/- 11 and 96 +/- 9 nmol/g fresh weight; of cholesterol 70 +/- 15 and 101 +/- 16; of cerebroside 19 +/- 3 and 41 +/- 7; of sulfatide 10 +/- 1 and 11 +/- l; and of gangliosides 0.80 +/- 0.08 and 0.40 +/- 0.05 N in cauda equina and spinal cord, respectively. The proportion of ethanolamine phosphoglyceride was lower and that of sphingomyelin higher in cauda equina than in spinal cord. The myelin of peripheral nerve and spinal cord contained almost the same proportions of lipids as the whole tissue. The protein-bound sialic acid content was 3-fold higher than the lipid-bound sialic acid content in cauda myelin. The fatty acid patterns of choline, ethanolamine, inositol and serine phosphoglycerides of spinal cord and its myelin, were very similar to those of cerebral white matter, while the phosphoglycerides of cauda equina had higher proportions of monoenoic acids and lower proportions of polyunsaturated fatty acids. The fatty acid patterns of sphingomyelin, cerebroside and sulfatide of spinal cord were similar to those of cerebral white matter, while those of cauda equina contained significantly more saturated fatty acids. This suggests that the lipid and fatty acid compositions of peripheral nerve are particularly suitable for the formation of a tightly packed myelin membrane which can be a powerful shield against infections and other injuries.

Adolescent↗
PubMed1980

Abnormal sulfate metabolism in a hereditary demyelinating neuropathy.

Trembler mice are affected by dominantly inherited neuropathy. Total lipid content and sulfatides were decreased in peripheral nerves from 15-day-old mutants. The proportion of sulfatides in per cent of total lipids was similar in control and Trembler nerves. The specific activity of ceramide galactosyltrnsferase, the enzyme responsible for the synthesis of cerebrosides, was 36 and 13% of controls, in young and adult. Trembler nerves, respectively. In contrast, cerebroside sulfotransferase activities were increased by 257 and 172% in young and adult Trembler sciatic nerves, respectively. No activator or inhibitor effect could be demonstrated. In Trembler PNS, Km, Vmax and heat sensitivity of CST differed from controls. Low levels of substrate and high arylsulfatase A activity (218% of controls) could explain the lack of sulfatide accumulation. The increased in vivo sulfate and galactose incorporation into non-lipidic material couild reflect the overproduction of endoneurial and perineurial connective tissue, whereas the high turnover rate of sulfatides could be correlated with intense demyelination and remyelination observed in Trembler PNS.

Animals↗
PubMed1981

Lipid metabolism and oxygen consumption in a hereditary demyelinating neuropathy, the trembler mouse: an in vitro study.

Sciatic nerves from 15-day-old trembler and control mice were maintained in vitro up to 53 h and the metabolism of myelin lipids and the oxygen consumption were investigated [35S]Sulfate was incorporated into sulfatides at a higher rate and turned over more rapidly in trembler nerves than in controls. [14C]Galactose was incorporated into cerebrosides of trembler nerves at a lower rate and turned over like the controls. In contrast, synthesis of sulfatides labeled with [14]galactose was increased in mutants and no significant turnover was observed for both trembler and control nerves during the whole incubation period. Similar results were obtained using [3H]serine as a precursor and no significant differences were observed in the turnover rates of sphingomyelin and phosphatidylcholine between trembler and control nerves. These data suggest the presence of two different pools of cerebrosides, a small one formed by the fast recycling of sulfatides and which does not mix with a second, larger one. The rate of oxygen consumption did not change significantly during the incubation period and was 2-3-fold higher in trembler nerves than in controls, reflecting, at least partly, the increased sulfatide metabolism.

Animals↗
PubMed1985

Metabolic alterations of endoneurial lipids in developing trembler nerve.

In vitro incorporation of [14C]acetate into desheathed sciatic nerve (endoneurium) was studied in developing normal and mutant trembler mice. The total uptake of [14C]acetate peaked 6 days after birth and decreased thereafter for both normal and trembler mice. Substantially less [14C]acetate (approximately 50% of normal) was incorporated in trembler mouse as early as 3 days after birth. In 3 and 6 day-old trembler mice, proportionately less [14C]acetate was incorporated into phospholipids and cerebrosides and more was incorporated into cholesterol. From 9 days, however, less [14C]acetate was incorporated into cholesterol of trembler compared to normal nerve. At later times (greater than or equal to 20 days), the proportion of [14C]acetate in both phospholipids and cerebrosides of trembler nerve was increased above that of controls. Throughout development, the relative incorporation of [14C]acetate into triacylglycerol and cholesteryl esters was about two times higher in trembler than in normal mice. In normal nerve, the 14C-label in free fatty acids increased (4-25%) progressively with age. In contrast, this incorporation in trembler nerves remained constantly low (less than 7%). Morphologically, the nerves of trembler mouse are markedly hypomyelinated. The abnormal myelin sheath undergoes cycles of breakdown and partial regeneration. Thus, the biochemical profile of extended active but perturbed lipid metabolism may provide a basis for the morphologic findings of continued active but ineffective myelination in trembler peripheral nerve.

Acetates↗
PubMed1986

Animal and cellular models of sphingolipid storage disorders of humans.

The synthesis of L-galactosylceramide is described. Data are presented indicating that this enantiomorph of D-galactocerebroside is not cleaved by galactocerebroside-beta-galactosidase obtained from mammalian tissues. The synthesis of L-glucosylceramide and beta-D-glucothiocerebroside are outlined. These compounds are also refractory to catabolism by glycosidases in mammalian tissues that catalyze the hydrolysis of naturally occurring cerebrosides. L-Hexosyl- and thioanalogs of cerebrosides and perhaps psychosines as well may be helpful for investigating the pathogenesis of Krabbe's disease and Gaucher's disease.

Animals↗
PubMed1980

The bile salt activation of leucocyte sphingolipid hydrolase activity and the modifying effects of triton X-100.

The activation of sphingomyelinase, galactosyl- and lactosylceramide beta-galactosidases, GM1, beta-galactosidase, cerebroside sulphate sulphatase, trihexosylceramide alpha-galactosidase and globoside beta-hexosaminidase by a number of bile salts was studied. Most of the salts examined, except glycolithocholate and deoxycholate, were effective activators of enzyme activity. Pure, but not crude, taurocholate elicited poor trihexosylceramide alpha-galactosidase and cerebroside sulphate sulphatase activities. The bile salt activation was often dependent on a number of parameters including the bile salt concentraion, pH, protein to bile salt ratio, and the nature of the enzyme preparation. In the absence of bile salts, Triton X-100 induced little activity of any of the hydrolases except sphingomyelinase; in their presence however, Triton either promoted or inhibited the activation depending on the enzyme. Our data suggest that bile salt structure is an important factor in determining the degree of activation of individual hydrolases and that this may be due to intramicellar lipid substrate/bile salt interactions, which either facilitate or inhibit the corresponding enzyme reaction.

Adult↗
PubMed1987

[D-Pen2,L-Pen5]enkephalin induced analgesia in the jimpy mouse: in vivo evidence for delta-receptor mediated analgesia.

Jimpy (B6CBA-A W-J/A-Ta jp) mice, which are known to be deficient in neuronal cerebroside sulfate (a putative component of the mu opioid receptor), were non-responsive in the tail flick test (compared to littermate controls and a standard Swiss strain of mouse) to analgesic doses of two mu opioid receptor agonists, morphine sulfate and [D-Ala2,MePhe4,Gly-ol5]enkephalin (DAGO). However, the jimpy mice responded normally (compared to controls) to the analgesic effects of the selective delta opioid receptor agonist [D-Pen2,L-Pen5]enkephalin (DPLPE). These results suggest (1) that delta opioid receptors can mediate analgesia and (2) that cerebroside sulfate is not necessary for delta opioid receptor activation.

Analgesics↗