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PubMed1968

Fatty acid composition of brain glycolipids in Alzheimer's disease, senile dementia, and cerebrocortical atrophy.

Quantitative fatty acid compositions are reported for human cerebral gray matter glycolipids (gangliosides, cerebrosides, and cerebroside sulfates) in Alzheimer's disease (presenile dementia) and related disorders of the central nervous system. Although the fatty acid compositions were generally similar to those of the controls, some significant differences were noted in Alzheimer's disease which were not found in the single samples of senile dementia and of senile cerebrocortical atrophy. In the ganglioside fatty acids, the percentage of palmitic acid was higher in all three cases of Alzheimer's disease than in the controls. In cerebrosides, the proportion of 2-hydroxy to non-hydroxy acids was slightly lower in Alzheimer's disease than in controls and the relative proportions of 2-hydroxystearic acid and 2-hydroxytricosenoic acid were slightly higher. In cerebroside sulfates, monounsaturated 2-hydroxy acids were lower in two of the samples with Alzheimer's disease. The results indicate possible effects of the degenerative changes in this disease on glycolipid metabolism.

Aged↗
PubMed1976

Quantitative analysis of brain galactosylceramides by high performance liquid chromatography of their perbenzoyl derivatives.

A high performance liquid chromatographic (HPLC) method for analysis of galactosylceramides as their benzyol derivates has been devised. Samples containing 10-150 nmoles of monohexoslceramides are benzoylated by heating for 60 min at 60 degree C in 0.5 ml of 10% (v/v) Benzoyl chloride in pyridine. The products are purified by solvent distribution and analyzed by SPLC. The benzoylated cerebrosides with nonhydroxy fatty acids are separated from those with hydroxy fatty acids on a Zipax column with 7% ethyl acetate in hexane as a solvent and UV absorption at 280 nm is recorded. This isocratic procedure can be applied directly to chloroform-methanol procedure can be applied directly to chloroform-methanol extracts of adult brain with a relative standard deviation of 3.0% for cerebrosides with nonhydroxy fatty acids and 4.0% for cerebrosides with hydroxy fatty acids. Sulfatides do not interfere in the assay and can be converted to cerebrosides after desulfation by mild acid methanolysis. Benzoylated glucosyl- and galactosylceramides can be separated on a MicroPak NH2 column with 1.5% 2-propanol in cyclopentane as the chromatographic solvent.

Animals↗
PubMed1983

Carbon monoxide-induced brain injury: neurochemical studies after single and repeated exposures.

Biochemical effects of a single and once-repeated exposure to carbon monoxide (0.2% CO, 30 min) were studied in mouse brain. The effect of the severity of the first exposure on the brain reaction to the second exposure (0.2% CO, 30 min) was also studied after exposure to four different CO doses (0.2% CO for 0, 2 or 5 h, and 0.3% CO for 2 h). 1. The DNA content of brain decreased after single and once-repeated CO exposures. The effect of the second exposure was greater than that of the first. 2. The changes in the glycosphingolipid synthesis of brain were measured as the specific rate of precursor incorporation into the two isolated galactolipid fractions, i.e., cerebrosides and gangliosides following a 2-h pulse-labelling with 3H-galactose in vivo. The synthesis of cerebrosides was substantially inhibited for a short period after the first and the second exposures. A late compensatory over-recovery of synthesis was found only after the second exposure. The rate of galactose incorporation into gangliosides was not immediately depressed after CO exposure, but otherwise the changes were similar to those of cerebrosides. 3. The protein, phospholipid and cerebroside contents of brain were also affected in parallel fashion with the changes of DNA content. The content of gangliosides was increased after the first, but not after the second exposure. The enhanced reaction of brain to the second CO poisoning suggests the existence of sublethal brain cell injury which is produced by the first CO exposure and manifested by altered reactions to the second exposure.

Animals↗
PubMed1978

Composition of lipids of bovine optic nerve.

Lipids from bovine optic nerve were analyzed. The total content of 16.5% by weight included 27.2% nonpolar lipids, 26.1% glycolipids, and 46.7% phospholipids by weight. Free cholesterol was the major component of the nonpolar lipid fraction. The cerebrosides, 73.5% of total glycolipids, were separated by thin layer chromatography (TLC) into two bands (upper and lower) that were present in equal proportion. Cerebroside sulfates comprised about 27.5% of total glycolipids. Gangliosides were also detected in the glycolipid fraction. In order of predominance, choline glycerophospholipids, ethanolamine glycerophospholipids, ethanolamine plasmalogens, serine glycerophospholipids, sphingomyelins, and inositol glycerophospholipids were the major phospholipids. Palmitoyl (16:0), stearoyl (18:0), and oleoyl (18:1) groups were the major acyl groups in all neutral and phospholipid classes. However, ethanolamine glycerophospholipids, serine glycerophospholipids, and inositol glycerophospholipids contained a large percentage of 22:6 (docosahexaenoyl) group. The major alk-1-enyl groups of the plasmalogens were 16:0, 18:0, and 18:1. Steroyl (18:0), lignoceroyl (24:0), and nervonoyl (24:1) were the major acyl groups in all sphingolipids. Lower cerebroside band and cerebroside sulfates contained large amount of hydroxylignoceroyl (cerebronoyl) and hydroxynervonoyl groups.

Animals↗
PubMed2006

Identification of complex mixtures of sphingolipids in the stratum corneum by reversed-phase high-performance liquid chromatography and atmospheric pressure photospray ionization mass spectrometry.

Sphingolipids, such as ceramides and cerebrosides, are important molecules in the formation and maintenance of the epidermal barrier to water vapor diffusion. In this paper we explore a new method to identify the sphingolipids found in the stratum corneum (SC), the outer layer of the epidermis, of House sparrows living in Saudi Arabia using reversed-phase high-performance liquid chromatography (HPLC) coupled with atmospheric pressure photo-ionization mass spectrometry (APPI-MS). First, using thin layer chromatography (TLC) we found that the SC contains ceramides, cerebrosides, and free fatty acids along with smaller amounts of cholesterol. Knowing the classes of sphingolipids present in the SC markedly reduced the number of possible molecules present. Then, we identified each sphingolipid molecule in our sample by both negative and positive mode of APPI-MS. We confirmed our identifications by generation of accurate mass data, and by examination of MS/MS spectra for selected molecules. Using APPI-MS, we identified 7 families of cerebrosides, for a total of 97 molecular species, and 4 families of ceramides, for a total of 79 molecules, in the SC of House sparrows, a wider array than would be found in mammals. Carbon chain lengths of fatty acids in the sphingolipids were longer than those that have been reported for mammalian SC; chain lengths of over 40 carbons were common. We also compared our estimates of the quantity of lipids in the SC obtained by HPLC/MS with those from TLC. Estimates of the amount of total ceramides and cerebrosides using TLC differed from those obtained by HPLC/MS by +0.95% and -2.5%, respectively. We conclude that our protocol using reversed-phase HPLC and APPI-MS is an useful method of analyzing complex mixtures of sphingolipids in the SC.

Animals↗
PubMed2005

Are glucocerebrosides the predominant sphingolipids in plant plasma membranes?

Long-chain sphingobases have been analyzed in various fractions prepared from different organs (leaf, root, storage tissue) from five dicotyledoneous plants (Arabidopsis thaliana, Brassica oleracea, Nicotiana tabacum, Pisum sativum, Spinacia oleracea). The resulting sphingobase profiles from cerebrosides and plasma membranes (PMs) show large qualitative and quantitative differences. Assuming that cerebrosides from all cellular membranes have similar sphingobase profiles, these data suggest that cerebrosides, considered to be characteristic glycolipids of plant PMs and specified by large proportions of sphingobases with an 8Z-double bond motif, do not represent the major sphingolipids of PMs. The fraction of unidentified complex sphingolipids, containing mainly 8E-phytosphingenine, exceeds the cerebroside proportion in PMs by several factors and may be as abundant as diacylglycerol-based phospholipids. These results are discussed with respect to the distribution of various lipids between the bilayer halves of plant PM.

Cell Membrane↗
PubMed1977

The biosynthesis of sulphogalactosyldiacylglycerol of rat brain in vitro.

Triton X-100 extracts of rat brain microsomal fraction catalyse the formation of sulphogalactosyldiacylglycerol from galactosyldiacylglycerol and adenosine 3'-phosphate 5'-sulphatophosphate. Of the various subcellular fractions of brain assayed, the microsomal fraction contained most (79%) of the adenosine 3'-phosphate 5'-sulphatophosphate-galactosyldiacylglycerol sulphotransferase activity. The enzyme activity was stimulated by Triton X-100 and showed linearity with increasing time, concentrations of enzyme and added substrates. ATP and KF prolonged the linearity of the activity with time, but ATP had an overall inhibitory effect on the sulphotransferase. Both ATP and KF inhibit the degradation of adenosine 3'-phosphate 5'-sulphatophosphate, which probably causes the increased linearity of the sulphotransferase reaction with time. The enzyme preparation did not catalyse the transfer of sulphate from adenosine 3'-phosphate 5'-sulphatophosphate to either cholesterol or galabiosyldiacylglycerol (galactosylgalactosyldiacylglycerol). Significant differences between the formation of sulphogalactosyldiacylglycerol and cerebroside sulphate catalysed by the same enzyme preparation were noted. ATP and Mg(2+) strongly inhibit the formation of sulphogalactosyldiacylglycerol but equally strongly stimulate the synthesis of cerebroside sulphate. The apparent K(m) for galactosyldiacylglycerol is 200mum, and that for cerebroside is 45mum. Galactosyldiacylglycerol and cerebroside are mutually inhibitory toward the synthesis of sulphated derivatives of each. These data do not necessarily lead to the conclusion that two sulphotransferases are present, but they do indicate a possible means of controlling the synthesis of these two sulpholipids.

Adenosine Triphosphate↗
PubMed1979

beta-Endorphin: formation of alpha-helix in lipid solutions.

Human beta-endorphin adopts a partial helical conformation in aqueous solutions of cerebroside sulfate, ganglioside GM1, phosphatidylserine, and phosphatidic acid, but not of cerebroside and phosphatidylcholine, as evidenced by circular dichroic spectra. Addition of Ca2+ to the peptide in cerebroside sulfate solution can break up the helix; at 10 mM Ca2+ the peptide (12 microM) essentially exists in an unordered form. For comparison, sheep beta-lipotropin in acidic cerebroside sulfate solution (pH less than 4) also has a partial helical conformation of the complex between human beta-endorphin and lipids may be related to the opiatelike function of this peptide hormone.

Adrenocorticotropic Hormone↗
PubMed1981

Prenatal diagnosis of Krabbe disease.

Krabbe disease was diagnosed prenatally in Göteborg (Sweden) and Lyon (France) by assaying the cerebroside-beta-galactosidase activity with galactosylceramides and lactosylceramides as substrates in cultivated amniotic fluid cells. Altogether, 48 pregnancies at risk were monitored between 1972 and 1980. Ten pregnancies at risk were terminated because of a predicted affection of the fetus. Biochemical examination of material available from 7 of the 10 abortuses confirmed the diagnoses. All the remaining 36 pregnancies ended in the birth of a healthy infant. The study showed that prenatal diagnosis of Krabbe disease is difficult because of the relatively high residual cerebroside-beta-galactosidase activity in some affected fetuses. Except for the large biological variation, the enzyme activity was sensitive to variation in cultivation conditions and differed strikingly between morphologically different cell types. These two factors were controlled by including control cell samples cultivated under identical conditions and by relating the cerebroside-beta-galactosidase activity to that of two marker enzymes. The biological variation was investigated further by measuring the cerebroside-beta-galactosidase activity in cultured skin fibroblasts from infants with Krabbe disease and from their parents. Results obtained in 18 unrelated patients with Krabbe disease, 26 obligate heterozygotes and 63 controls showed a wide range of variation in enzyme activity in the controls, a large overlap between the controls and obligate heterozygotes, and a high residual activity in some patients. Nevertheless, a high residual activity in a patient was combined with a relatively high enzyme activity in the two parents. In the light of the above findings and deliberations, it appears warranted to conclude that laboratories with experienced personnel can make a reliable prenatal diagnosis of Krabbe disease and that the examination should be offered to all known couples at risk.

Cells, Cultured↗
PubMed1988

A survey of neurological mutant mice. I. Lipid composition of myelinated tissue in known myelin mutants.

The lipids of white matter and peripheral nerve from mutant mice with known myelin deficiencies were analyzed by one- and two-dimensional high-performance thin-layer chromatography and quantitated by densitometry. In optic nerve, the mutants jp/Y, jpmsd/Y, qk/qk, shi/shi and shimld/shimld, which have severe central nervous system (CNS) myelin deficiency, had a common pattern of lipid loss: cerebrosides and sulfatides (hydroxy and nonhydroxy forms) were generally reduced by 70-95% or more; most phospholipids were diminished by 15-55%, and cholesterol was reduced by 35-60%. Only in the CNS of jp/Y and jpmsd/Y did cholesterol ester accumulate. In peripheral nerve, the lipid composition varied markedly among these mutants. In jp/Y there was no change, while in jpmsd/Y there was a 5-15% loss among the phospholipids and cholesterol. Homozygous qk had reductions of 75-85% in the nonhydroxy forms of cerebroside and sulfatide, a 130% increase in hydroxy sulfatide, and a 55% loss of sphingomyelin. In shi/shi and shimld/shimld homozygotes, the glycolipids were altered by +/- 20%, most phospholipids and cholesterol were reduced by 5-15%, and sphingomyelin was reduced by 40%. Tr and TrJ showed 35-90% reductions in most lipid classes of the peripheral nervous system; CNS lipid composition was normal. Homozygous twi had a uniform loss of most lipid classes in both optic (generally 10-20%) and trigeminal nerves (generally 40-55%); cerebrosides did not accumulate in these tissues. dy/dy had a 10-20% reduction of cerebrosides in trigeminal nerve trunk. The CNS of dy homozygotes had 10-35% increases in specific classes of glycolipids and phospholipids, and in cholesterol. None of the mutants showed detectable levels of lysophospholipids or other unusual lipid species. The fractions of ethanolamine and choline phosphatides in the plasmalogen form were close to normal in all mutants.

Animals↗
PubMed2000

Characterization of UDP-glucose:ceramide glucosyltransferases from different organisms.

Cerebrosides are typical membrane lipids of many organisms. They occur in plants, fungi, animals, humans and some prokaryotes. Almost all of our knowledge on the physiological functions of cerebrosides results from experimental data obtained with mammalian cells. However, very little is known about the roles played by these lipids in plants and fungi. To initiate such investigations we have cloned and characterized a ceramide glucosyltransferase from the yeast Candida albicans. Functional expression of this gene in Saccharomyces cerevisiae led to the accumulation of new glycolipids which were not present in wild-type baker's yeast. They were identified by MS and NMR spectroscopy as beta-D-glucopyranosyl ceramides. The ceramide moieties of these cerebrosides comprised phytosphinganine and mainly long-chain (C(26)) alpha-hydroxy fatty acids in amide linkage. We also generated a ceramide glucosyltransferase-knock-out strain of C. albicans which was devoid of cerebrosides. The viability of this mutant showed that for this organism glucosyl ceramides are not essential for vegetative growth on complete or minimal media. In addition, we have cloned and functionally expressed one of the three putative glucosylceramide synthases from Caenorhabditis elegans, as well as a corresponding enzyme from Pichia pastoris.

Candida albicans↗
PubMed1986

Stimulatory effect of certain plant sphingolipids on fruiting of Schizophyllum commune.

We reported previously that certain cerebrosides and ceramides from fungi were active upon fruiting of Schizophyllum commune (Kawai, G., and Ikeda, Y. (1982) Biochim. Biophys. Acta 719, 612-618; Kawai, G., and Ikeda, Y. (1983) (Biochim. Biophys. Acta 754, 243-248). This work was undertaken to extend our study to sphingolipids in wheat grain. The cerebrosides from wheat grain were fractionated by high-performance liquid chromatography into at least 40 components with and without the fruiting-inducing activity. Four major active fractions were characterized by thin-layer chromatography, infrared spectroscopy, gas-liquid chromatography, gas-liquid chromatography-mass spectroscopy, and 1H and 13C nuclear magnetic resonance spectroscopy. The active cerebrosides consist of glucose, 2-hydroxyhexadecanoic acid or 2-hydroxyoctadecanoic acid acid, and (4E,8Z)-sphingadienine or (8Z)-sphingenine. The cerebroside with (8Z)-sphingenine became inactive when the double bond was hydrogenated. Diglycosylceramides were as active as the monoglycosylceramides, but triglycosylceramides were only about 10% as active. The relationship between the structure and the activity is discussed.

Basidiomycota↗
PubMed1974

Glycosphingolipids from rabbit aorta, plasma, and red blood cells: effects of high cholesterol-high fat diets on fatty acid distribution and quantity of glycosphingolipids.

Four glycosphingolipids were isolated from rabbit aorta, plasma, and red blood cells. They were identified, by thin-layer chromatography and by quantitative analysis of hexose and fatty acid, as cerebroside, diglycosyl ceramide, triglycosyl ceramide, and globoside. The rabbits had been maintained on a normal diet or on one of three high cholesterol diets for 180 days. The quantities of the glycosphingolipids and their fatty acid distributions were determined, and comparisons were made between the control and experimental animals. Aorta and plasma glycosphingolipids were more affected by the high cholesterol diets than were those from red blood cells. The effects on aorta and plasma glycosphingolipids were similar. The amount of cerebroside was increased in aorta and plasma in all animals in the experimental groups. The amount was also increased in red blood cells in rabbits from two of the experimental groups. The average fatty acid chain length was greater in the lipids from the experimental animals than in those from the control animals for all measured glycosphingolipids from aorta. The average chain length was also greater in cerebrosides from the experimental animals from all three tissues. Probably the most notable differences in the experimental animals were the increased 24:1/24:0 ratios and the increased concentrations of 24:2. These increases occurred in nearly all samples from plasma and aorta, but not in red blood cells. There was also an increase of total unsaturated fatty acids in aorta cerebrosides from the experimental animals. Except for the increase in 24:2, lard generally caused more deviation from normal than did cottonseed oil when the level of cholesterol in the diet was 1%.

Animals↗
PubMed1978

Studies on the function of the activator of sulphatase A.

The activator of sulphatase A is necessary for the enzymic degradation of sulphatides to cerebrosides at ionic concentrations in the physiological range (1). Activation is probably due to the reversible formation of a one-to-one complex between activator and sulphatides (1,2). Formation of this complex is partly inhibited by cerebrosides due to competitive binding (2), as well as by phospholipids (e.g. lecithin or phosphatidylserine). Inhibition of the complex formation between activator and sulphatides by cerebrosides and phosphatidyl-serine depends on the concentration of the lipids and is of the same order of magnitude as the inhibition (by these lipids) of the enzymic degradation of sulphatides in the presence of activator (1). Moreover the degradation rate of sulphatides increases with the concentration of activator-sulphatide complex in the reaction mixture (1) indicating that the activator-sulphatide complex is the substrate for the enzyme in the degradation of sulphatides by sulphatase A.

Cerebroside-Sulfatase↗
PubMed1979

Nerve lipid abnormalities in human diabetic neuropathy: a correlative study.

To study endoneurial lipid composition in human diabetic neuropathy, we biopsied sural nerves from 3 middleaged men with adult-onset diabetes mellitus. Magnitude of electrophysiological abnormalities and myelinated fiber loss paralleled the clinical severity of neuropathy in all cases. Cholesterol ester concentration was elevated to about 800% of normal in diabetic nerves. Reduction in total endoneurial lipid concentration correlated best with decrease in myelin volume as calculated from measured fiber diameters. Cholesterol, cerebroside, and most phospholipids were reduced in keeping with the severity of fiber loss in each nerve. The phosphatidylinositol-phosphatidylserine fraction was most reduced in the least affected nerves. Cerebroside nonhydroxy fatty acids in diabetic nerves were of shorter chain length and more saturated than normal. It is not yet clear whether the abnormalities of phosphatidylinositol-phosphatidylserine and cerebroside fatty acids are of pathogenetic importance or whether these changes may be the nonspecific consequence of axonal degeneration.

Adult↗
PubMed1983

Prenatal diagnosis of pseudo arylsulphatase A deficiency.

Prenatal diagnosis was performed on a pregnancy at risk for metachromatic leukodystrophy (MLD) in a family with the pseudo arylsulphatase A deficiency trait. Extracts of cultured amniotic fluid cells were deficient in arylsulphatase A indicating that the fetus was either affected with MLD or had the benign pseudodeficiency trait. In the cerebroside sulphate loading test, the at risk cells hydrolysed sulphatide like control cultured amniotic fluid cells implying that the fetus had pseudodeficiency. The pregnancy was carried to term and a male child was delivered. Placenta, urine and fibroblasts had very low activities of arysulphatase A. However, no sulphatide could be detected in urine and growing fibroblasts responded normally in the cerebroside sulphate loading test, suggesting pseudodeficiency. At 29 months, the infant is healthy and shows no stigmata of MLD. The prediction based on the results of the cerebroside sulphate loading test on cultured amniotic fluid cells appeared to be borne out.

Amniotic Fluid↗
PubMed1980

Lipids of myelin, white matter and gray matter in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation.

The lipid classes and fatty acid compositions of myelin, white matter and gray matter were analyzed in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation. When compared with normal data, the percentage of 24:1 was considerably decreased and diminished unsaturation was observed in cerebrosides, whereas the sum of 24:0 and 24:1 was the same as in normals. The ratio of hydroxy fatty acids to total fatty acids in cerebrosides was low. The contents of cholesterol and phospholipids in white matter were reduced to 80% of the normal, whereas cerebroside was reduced to 48% of the normal.

Brain Chemistry↗
PubMed1983

Hydration of N-palmitoylgalactosylsphingosine compared to monosaccharide hydration.

Differential scanning calorimetry (DSC) studies of the ice-water transition of N-palmitoylgalactosylsphingosine (NPGS) (cerebroside)/water mixtures indicate 4 +/- 1 non-freezable water molecules per molecule NPGS. This hydration level, representing strongly bound water, is identical to that observed previously for human glucocerebroside (Bach, D., Sela, B. and Miller, I.R. (1982) Chem. Phys. Lipids 31, 381). Comparison of gluco- and galacto-cerebroside hydration with hydration measurements on simple monosaccharides suggests a favored orientation of the glycosyl polar group at the cerebroside-water interface.

Animals↗