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PubMed1986

Effect of antiglycolipid antisera on the lipid composition of cultured mouse spinal cords.

To examine the effect of antiglycolipid antibodies on demyelination, myelinated cultures of embryonic mouse spinal cords were treated by antigalactocerebroside (anti-GC), anti-GM1; and anti-GM4 antisera, and the lipid composition of the cultures were studied. The anti-GC antiserum-treated cultures, which exhibited severe morphologic signs of demyelination, revealed a significant reduction of cerebroside. The anti-GM4 or anti-GM1 antiserum-treated cultures, which exhibited mild degrees of demyelination, also had low contents of cerebroside. These results support our previous data showing that antiglycolipid antibodies cause demyelination in cultured mouse spinal cords, and suggest a possible role of myelin-specific glycolipids in the demyelination process.

Animals↗
PubMed1987

Fecal excretion of intestinal glycosphingolipids by newborns and young children.

Glycosphingolipids were shown to persist in human fecal excretions from birth up 2 years of age. The pattern of glycosphingolipids was dependent on blood group and secretor status of the child and changed dramatically during the first months of life. Perinatally cerebroside, hematoside and blood group active fucolipids were dominating among fecal glycolipids. From the time of weaning lactosylceramide abruptly became and then persisted as a dominating glycolipid although cerebroside, complex gangliosides and blood group active fucolipids could still be detected in feces even at 2 years of age.

Age Factors↗
PubMed1985

Brain myelin of genetically obese mice.

Brain myelin was studied in genetically obese (ob/ob) mice and compared with that of normal (+/+) mice from the same strain. The brain from obese mice had a significantly lower amount of myelin, and marked changes in the fatty acid composition of myelin were observed. In contrast, the myelin cholesterol: phospholipid: galactolipid and the cerebroside: sulfatide molar ratios and also the phospholipid composition were normal. 2',3'-Cyclic nucleotide 3'-phosphohydrolase (CNP) and UDP-galactose ceramide galactosyltransferase (CGalT) enzymatic activities were normal, and 3'-phosphoadenosine 5'-phosphosulfate (PAPS): cerebroside sulfotransferase (CST) was only slightly decreased. Cholesterol esters were not present in the mutant. These results indicate that brain myelination is affected in obese mutant mice.

2',3'-Cyclic Nucleotide 3'-Phosphodiesterase↗
PubMed1989

Buoyant density and lipid composition of purified myelin of aging human brain.

Purified myelin of human brain from 15 young adult (below 50 years of age) and old (above 70 years of age) autopsy cases each was examined by isopycnic centrifugation in continuous sucrose gradients, and for lipid composition. The mean buoyant density of myelin was the same in both groups. Apparent features of old age were a wide range of density values, less compact myelin bands, and the dissociation of myelin into two bands in six of 15 old cases. Lipid analyses of randomly selected myelin samples of each group revealed an inverse relationship between the total lipid to protein ratio and density of myelin. In old age total lipids decreased by an average 10 mol lipid per mol protein. This decrease was accounted for by cholesterol, phosphatidylserine and cerebrosides. Changes in fatty acid moieties mainly affected sphingolipids. C20:0 and C24:0 of sphingomyelin increased, as did even more markedly the more hydrophilic OH-fatty acids of cerebrosides. Correlations with buoyant density existed for the ratios of cholesterol to protein in young adult cases, and those of galactolipids to protein in old cases. The results suggest that old age is associated with impaired stability and altered lipid composition of myelin.

Adolescent↗
PubMedDate not supplied

Interaction of bradykinin with sodium dodecyl sulfate and certain acidic lipids.

The striking change in the circular dichroism (CD) of bradykinin (BK) occasioned by its interaction with sodium dodecyl sulfate (SDS) is evidently due in large part to a change in the conformation of the C-terminal tetrapeptide moiety of the hormone. The full change in CD is induced by the binding of two molecules of monomeric SDS per peptide molecule, the complex being aggregated. Formation of the 1:2 BK-SDS complex apparently proceeds via intermediates of stoichiometry 1:1 and 2:1. The cooperative nature of the interaction is attributed to the SDS-promoted aggregation of BK. Electrostatic interactions with the Arg residues appear important for the binding reaction per se. CD reveals that BK also interacts with acidic lipids which bear a net electrical charge (e.g., cerebroside sulfate and phosphatidyl inositol) but not with lipids bearing no net charge (e.g., cerebroside and phosphatidyl choline). The interactions are with particular mixed micelles of the lipid and the nonionic surfactant used for their solubilization, micellar size and structure being examined by surface tensiometry and electron microscopy.

Bradykinin↗
PubMed1983

Galactocerebroside-phospholipid interactions in bilayer membranes.

Differential scanning calorimetry (DSC) and x-ray diffraction have been used to study the interaction of hydrated N-palmitoylgalactosylsphingosine (NPGS) and dipalmitoylphosphatidylcholine (DPPC). For mixtures containing less than 23 mol% NPGS, complete miscibility of NPGS into hydrated DPPC bilayers is observed in both the bilayer gel and liquid-crystal phases. X-ray diffraction data demonstrate insignificant differences in the DPPC-bilayer gel-phase parameters on incorporation of up to 23 mol% NPGS. At greater than 23 mol% NPGS, additional high-temperature transitions occur, indicating phase separation of cerebroside. For these cerebroside concentrations, at 20 degrees C, x-ray diffraction shows two lamellar phases, hydrated DPPC-NPGS gel bilayers (d = 64 A) containing 23 mol% NPGS, and NPGS "crystal" bilayers (d = 55 A). On heating to temperatures greater than 45 degrees C, the mixed DPPC-NPGS bilayer phase undergoes chain melting, and on further increasing the temperature progressively more NPGS is incorporated into the liquid-crystal DPPC-NPGS bilayer phase. At temperatures greater than 82 degrees C (the transition temperature of hydrated NPGS), complete lipid miscibility is observed at all DPPC/NPGS molar ratios.

Calorimetry↗
PubMed1992

Calorimetric and x-ray diffraction studies of rye glucocerebroside mesomorphism.

Glucocerebrosides (GlcCer) isolated from the leaves of winter rye (Secale cereale L. cv Puma) differ from the more commonly investigated natural and synthetic cerebrosides, in that greater than 95% of the fatty acids are saturated and monounsaturated hydroxy fatty acids. Isomers of the trihydroxy long chain base hydroxysphingenine (t1(8:18 cis or trans)) and isomers of sphingadienine (d18:2(4trans, 8 cis or trans)) comprise 77% and 17%, respectively, of the total long chain bases. The phase behavior of fully hydrated and dry rye leaf GlcCer was investigated using differential scanning calorimetry (DSC) and x-ray diffraction. On initial heating, aqueous dispersions of GlcCer exhibit a single endothermic transition at 56 degrees C and have an enthalpy (delta H) of 46 J/g. Cooling to 0 degrees C is accompanied by a small exothermic transition (delta H = -8 J/g) at 8 degrees C. On immediate reheating, a broad exothermic transition (delta H = -39 J/g) is observed between 10 and 20 degrees C in addition to a transition at 56 degrees C. These transitions are not reversible, and the exothermic transition rapidly diminishes when the sample is held at low temperature. Using x-ray diffraction, it was determined that the endotherm at 56 degrees C represents a transition from a highly ordered lamellar crystalline phase (Lc) with a d-spacing of 57 A and a series of wide-angle reflections in the 3-10 A range, to a lamellar liquid crystalline (L alpha) phase having a d-spacing of 55 A and a diffuse wide-angle scattering peak centered at 4.7 A. Cooling leads to the formation of a metastable gel phase (L beta) with a d-spacing of 64.0 A and a single broad reflection at 4.28 A. Subsequent warming to above 15 degrees C restores the original Lc phase. Thus, rye GlcCer in excess water exhibit a series of irreversible transitions and gel phase metastability. Dry GlcCer undergo an initial heating endothermic transition at 130 degrees C, which is ascribed to a transformation into the HII phase from a two phase state characterized by the coexistence of phases with disordered (alpha) and helical (delta) type chain conformations but of unknown lattice identity: An exotherm at 67.5 degrees C observed upon subsequent cooling is of unknown origin. Since an undercooled HII phase persists down to 19 degrees C, the exotherm may derive in part from an alpha-to-delta type chain packing conformational change especially under slow cooling conditions. Upon reheating from low temperatures to 65 degrees C, a phase with a two-dimensional, primitive rectangular lattice and delta-like chain packing (R8 phase) in coexistence with the HI, phase emerges. With continued heating to 90 degrees C these coexisting phases give way to a phase with a two-dimensional, centered rectangular lattice and delta-like chain packing (P8phase) which again coexists with the HI, phase. Above 130 degrees C, the Pb phase disappears and the sample converts completely to the HI, phase as observed upon initial heating. These results indicate that the mesomorphic behavior of rye leaf GIcCer is distinct from that of other cerebrosides.

Biophysical Phenomena↗
PubMedDate not supplied

Croatian population data for arylsulfatase a pseudodeficiency-associated mutations in healthy subjects, and in patients with Alzheimer-type dementia and Down syndrome.

BACKGROUND: Arylsulfatase A (ASA) is a lysosomal enzyme involved in catabolism of cerebroside sulfate, whose deficiency causes metachromatic leukodystrophy, a rare autosomal recessive disorder characterized by storage of cerebroside sulfate, mainly in the nervous system. Low ASA activities have also been reported in healthy individuals and several neuropsychiatric disorders due to the condition termed ASA pseudodeficiency. The aim of this study was to establish frequency of two mutations associated with ASA pseudodeficiency in healthy individuals in the Croatian population as well as in persons with Alzheimer-type dementia and Down syndrome. METHODS: Presence of N350S and 1524+95 A-->G pseudodeficiency mutations was detected in genomic DNA extracted from leukocytes of healthy subjects (n = 125) and of patients with Alzheimer-type dementia (n = 18) and Down syndrome (n = 21). Arylsulfatase A activity was measured in leukocyte homogenates by spectrophotometry (lambda = 515 nm) using p-nitrocatechol sulfate as chromogenic substrate. RESULTS: Frequency of N350S mutation and mutation 1524+95 A-->G was estimated at 6.8 and 2.8% for healthy controls, 11 and 5.5% for Alzheimer-type dementia, and 12 and 9.5% for Down syndrome, respectively. Arylsulfatase A activity was slightly but not significantly decreased in leukocytes derived from subjects with dementia and Down syndrome in comparison with age-matched control samples. CONCLUSIONS: Frequency of two mutations associated with ASA pseudodeficiency in the Croatian population is slightly below the range reported for other populations. Additionally, despite the proposed role of arylsulfatase A pseudodeficiency as one of the predisposing factors for neuropsychiatric disorders, our preliminary results did not show significantly higher frequencies of either mutation in Alzheimer-type dementia or Down syndrome.

Adolescent↗
PubMed1990

Infantile osteopetrosis complicating neuronal ceroid lipofuscinosis.

An autopsy case of infantile osteopetrosis complicating neuronal ceroid-lipofuscinosis is reported. Autopsy revealed generalized sclerosis and thickening of cortical and spongy bones, formation of mineralized cartilagenous tissues, and narrowing of the marrow cavities associated with decreased hematopoietic cell components. Around the thickened bone trabecles, osteoclasts lacked a ruffled border and clear zone along the cell membrane facing the bone matrix surface. The brain was markedly atrophic with neuronal cell loss and focal gliosis, and the remaining neuronal cells accumulated brown granular pigments, which were confirmed histochemically and electron-microscopically to be ceroid and lipofuscin. In the cerebral medulla, the development of myelin sheaths was extremely poor. Also, the occurrence of Lex type glycolipids and GM3 and the apparent absence of cerebroside and cerebroside sulfate were proved by biochemical analysis, suggesting that the brain was still in a stage of embryonic development or still in the process of differentiation. Except for one suggestive case, this is the first case of complicating neuronal ceroid-lipofuscinosis in infantile osteopetrosis.

Atrophy↗
PubMed1992

Novel modification of glycosphingolipids by long-chain cyclic acetals: isolation and characterization of plasmalocerebroside from human brain.

A glycosphingolipid component of human brain, having long-chain cyclic acetals, has been isolated and characterized. This compound incorporates a novel type of natural glycan modification, in which a long-chain aliphatic aldehyde is conjugated through a cyclic acetal (plasmal) linkage to the galactosyl moiety of cerebroside. In addition to components normally observed by gas chromatography-mass spectrometry (GC-MS) following methanolysis of cerebroside (fatty acid methyl esters, methyl alpha- and beta-galactosides, sphingosine), this compound produced 16:0, 18:0, and 18:1 fatty aldehydes, unequivocally identified as their enol methyl ether derivatives. Results of positive ion fast atom bombardment mass spectrometry (FAB-MS) of the native compound, and GC-MS of partially methylated hexitol acetates derived from the permethylated derivative, were consistent with structures of galactocerebroside having 3,4- and 4,6-linked cyclic plasmal substituents, as shown. [formula: see text]

Acetals↗
PubMed1976

Sulfated glycoproteins, glycolipids, and glycosaminoglycans from synaptic plasma and myelin membranes: isolation and characterization of sulfated glycopeptides.

In this report we provide biochemical evidence that a highly purified synaptic plasma membrane fraction derived from rat brain, after intraventricular injection of 35S-labeled sodium sulfate, is enriched in a number of large sulfated glycoproteins compared with a purified myelin fraction studied concurrently. A fraction of the detergent-solubilized sulfated glycoprotein bound specifically to concanavalin A-Sepharose. In addition, we have identified the 35S-labeled lipid-soluble material in these membrane fractions as cerebroside sulfate. The sulfated protein in the lipid-extracted membranes was shown to consist predominantly of a class of glycoproteins containing sulfate in ester linkage to oligosaccharide chains, which are differentiated structurally from the sulfated glycosaminoglycans of brain. These two classes of sulfated macromolecules were distinguished from one another by several chemical and physical parameters. We present the chemical characterization of the sulfated glycopeptides derived from synaptic plasma and myelin membranes by extensive proteolytic digestion after quantitative removal of cerebroside sulfate. Membrane-associated glycosaminoglycans, either specifically or adventitously associated with these neuronal membranes, were quantitatively precipitated and identified.

Amino Acids↗
PubMed1968

The lipid composition of rat brain myelin and subcellular fractions during development.

1. The lipids of whole brain and subcellular fractions of the rat were analysed during development. 2. The deposition of cholesterol occurred in two phases, one related to increasing wet weight of the brain and the second to myelination. Cerebroside accumulation was related only to myelination. 3. The composition of myelin isolated from 12-day-old rat brain was different in some respects from that of the adult. In the former there was an increase of phospholipid in relation to cholesterol and a marked deficiency in cerebroside. 4. It is suggested that early myelin is extruded glial plasma membrane, which only later becomes mature myelin.

Aging↗
PubMed1985

Late-onset globoid cell leucodystrophy (Krabbe's disease). Clinical and genetic delineation of two forms and their relation to the early-infantile form.

A girl is described with a late-onset form of globoid cell leucodystrophy (GLD, Krabbe's disease). Data of this patient and seventeen reported patients with late-onset GLD and cerebroside-beta-galactosidase deficiency were compared with those of patients with classical early-infantile GLD. Three phenotypes of GLD are proposed, an early-infantile form, and two late-onset forms. Biochemical studies demonstrated residual activities of cerebroside-beta-galactocerebrosidase in the late-onset forms. The KM values were identical in the three GLD phenotypes. Autosomal recessive inheritance is likely for each of the subtypes. Complementation studies by somatic cell hybridization suggest that the mutations in early-infantile and late-onset GLD are allelic.

Cerebrosides↗
PubMed1976

Postnatal patterns of brain lipids in progeny of vitamin B-6 deficient rats before and after pyridoxine supplementation.

The influence of deficient and adequate maternal intakes of pyridoxine on lipid profiles in brains of progeny at 5, 10, 15, 25 and 50 days of age was studied. The effects of supplementing deficient dams at two different times with pyridoxine on the brain development of progeny were also examined. Three groups of weanling, female rats were fed diets deficient in pyridoxine (1.2 mg pyridoxine-HC1/kg diet) and another group received a control diet (30.0 mg pyridoxine-HC1/kg diet). One deficient group and the control group were fed their diets throughout growth, gestation and lactation. Two groups of dams were fed the deficient diet through growth, gestation and until 5 or 10 days postpartum when pyridoxine was supplemented by feeding the control diet. Body and brain weights were significantly lower in 15, 25 and 50 day-old progeny of deficient dams and deficient dams supplemented at 10 days postpartum. Cerebroside content at 15 days and ganglioside content at 15 and 25 days were significantly lower in brains of pups from unsupplemented deficient dams and deficient dams supplemented at 10 days postpartum. The postnatal development of cerebroside and ganglioside levels in brain was delayed or retarded in brain of pups from unsupplemented deficient dams. Supplementation of dams fed a low level of pyridoxine (1.2 mg/kg diet) with the vitamin beginning at 5 days postpartum reversed all observed effects of the low vitamin intake on brain lipids in progeny.

Age Factors↗
PubMed1983

The effect of long-term low protein intake on lipids of rat brain during adulthood.

The effect of experimental protein malnutrition on growth, brain tissue weight and lipid composition was studied in male Wistar rats fed isoenergetic diets containing 22% (C-22) or 6% protein (PD-6) from 60 days until 240 days of age. At 240 days of age the mean weight of PD-6 rats was 63% that of control rats. Protein malnutrition initiated at 60 days of age affected lipid composition by 240 days of age. The total content of phospholipids and the phosphatidylethanolamine, phosphatidylcholine, phosphatidylserine and phosphatidylinositol fractions were not significantly changed in rats ingesting the PD-6 diet, but phosphatidylglycerol and sphingophospholipid fractions were significantly decreased in the brains of PD-6 rats. Such a diet did not cause a statistically significant decrease of the total amount of monogalactosyl glycolipid or of most investigated fractions (cerebrosides with hydroxy fatty acids, sulfoglycolipids and monogalactosyldiacylglycerols), except for cerebrosides with nonhydroxy fatty acids. Long-term, low protein intake did not cause a significant decrease in the total ganglioside content, but had an effect on the composition of single investigated ganglioside fractions (GM1, GD1a, GD1b and GT1a).

Animals↗
PubMed1976

Chemical compositions of brain and myelin in two patients with multiple sulphatase deficiency (a variant form of metachromatic leukodystrophy).

The lipid composition of the brain, including myelin, was studied in detail in two cases with a variant form of metachromatic leukodystrophy (multiple sulphatase deficiency type). In the white matter, the sulphatide concentration was 3-4 times higher than the normal level in both cases. There was a significant accumulation of cholesterol sulphate in the brain, liver and kidney of both cases. The ganglioside pattern in the grey and white matter was abnormal, with a higher proportion of GM3, GM2 and GD3-gangliosides. Non-lipid hexosamine contents were increased 1.5-2 times in brain, 8-10 times in liver and 2-3 times in kidney. Increased amounts of glucocerobroside, ceramide lactoside and ceramide trihexoside were present in grey and white matter of both cases. Recovery of purified myelin from two patients' brains was much less than from control (1-2% in case 1 and 20-30% in case 2). The lipid composition of myelin was almost normal except for a higher proportion of sulphatide, with a decreased amount of cerebroside. The fatty acid compositions of myelin sulphatide and sphingomyelin were almost normal, while non-hydroxy fatty acids of cerebroside contained less long-chain fatty acids, as characterized by a significant increase of C16:0 and C18:0 fatty acids. The myelin polypeptide pattern by SDS-disc gel electrophoresis showed a relative decrease of basic protein and of proteolipid protein. A possible mechanism of myelin loss in MSD is discussed.

Antigens, CD↗
PubMed1980

Chronic alcohol intake and some monogalactosyl glycolipids of the rat brain.

The effect on monogalactosyl glycolipid complement (cerebrosides with hydroxy and non-hydroxy fatty acids, sulphatides and monogalactosyldiglyceride) after 38 weeks of feeding 25% sucrose-32% ethyl alcohol have been studied. Chronic alcohol intake produced a statistically significant increase of cerebrosides with hydroxy and non-hydroxy fatty acids and monogalactosyldiglycerides as well as decrease of sulphatides in male Wistar rats brain.

Animals↗
PubMed1981

Effect of triethyl tin on myelination in the developing rat.

Myelinogenesis in developing rats was studied following chronic dosing with triethyl tin (TET), at a level of 1.0 mg TET/kg body wt/day. Experiments included starved controls with body weights depressed by 17 to 40% to equal those of the TET-treated groups. Rats at ages of 16, 21, and 30 days showed decreases relative to well-nourished controls in body weight, forebrain weight, myelin yield, cerebroside level, and specific activity of brain 2',3'-cyclic nucleotide-3'-phosphohydrolase when dosed with TET. At 30 days, myelin and cerebroside yields were reduced by approximately 55%, while CNP activity was reduced by less than 20%. No differences in the forebrain myelin protein composition between control, starved, and TET animals were noted. The rate of myelin protein synthesis relative to brain total protein (assayed by incorporation of intracranially injected [3H]glycine into brain homogenate and myelin proteins) was decreased in the TET rats in proportion to the decreased yield of myelin, but no particular myelin protein was preferentially affected. Matching starved controls exhibited similar body weight decreases, less pronounced forebrain weight decreases, and little or no decrease in myelin concentration. There was a relative increase in the myelin protein synthesis rate in the starved rats, indicating preferential utilization of limited protein precursors for myelin protein synthesis. Spinal cord myelin was also decreased in the TET rats, but less severely than in the forebrain. At all ages optic, but not sciatic, nerves showed decreases in myelin concentration with TET treatment. We conclude that TET inhibits forebrain growth and CNS myelination more severely than can be accounted for by a general metabolic insult.

Animals↗