Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “CEREBROSIDES”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 757 records · Page 42Linked to original sources

Metamorphic changes in glycolipids and myelin proteins and 2',3'-cyclic nucleotide 3'-phosphohydrolase in bullfrog and axolotl brains.

The metamorphic changes in levels of glycolipids and myelin proteins and 2',3'-cyclic nucleotide 3'-phosphohydrolase (CNP) in the brains of bullfrog tadpoles, adult frogs, and axolotls were investigated, with particular emphasis on myelin maturation. The concentrations of cerebroside, sulfatide, and galactosyldiacylglycerol gradually increased from the onset of prometamorphosis throughout the active metamorphic period and then greatly increased after metamorphosis was completed. The ratio of glucocerebroside to galactocerebroside increased greatly in the prometamorphic period and then rapidly decreased to the frog level during the climax period. The fatty acid compositions of cerebroside and sulfatide showed a developmental change, with 24:1 being more predominant in the later metamorphic stage. The proportion of hydroxy fatty acids increased up to the onset of the prometamorphic stage and thereafter remained constant at approximately 50% of the total. The CNP activity remained unchanged throughout metamorphosis at 60% that in frog myelin and increased in the adult frog. The composition of tadpole myelin proteins remained constant during metamorphosis, with large basic protein being the most abundant, and in the frog, proteolipid protein and large basic protein were present in comparable amounts. The two adult forms of axolotl, i.e., the neotenous and metamorphosed forms, exhibited almost identical myelin constituents, and CNP activity in the neotenous form amounted to one-fifth that in the bullfrog. These results indicate that active biosynthesis of myelin marker components occurs as metamorphosis proceeds, but more pronounced changes of myelin components occur after metamorphosis is completed.

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

Tubules of globoid leukodystrophy: a right-handed helix.

Morphologic similarity between the cytoplasmic tubules of globoid leukodystrophy and Gaucher's disease (as demonstrated by thin sectioning, negative staining, and shadowing techniques) and their resemblance to negatively stained beef cerebroside are presented as evidence favoring the accumulation of cerebrosides in globoid cells. The tubules of globoid leukodystrophy have a 60-angstrom periodic banding similar to the tubules of Gaucher's disease. A right-handed helical twisting of the tubules is observed in both diseases.

Animals↗

Lipid composition and activity of a lytic factor isolated from Plasmodium berghei.

A fraction was obtained from Plasmodium berghei which induced hemolysis of the erythrocytes of mice and hamsters. This fraction, called lytic factor (LF), was found to be composed of a large amount of lipid material. An examination of the lipids showed the major lipids to be monoglycerides, diglycerides, triglycerides, fatty acids, long-chain alcohol, sterol, sterol ester, sterol glycoside, and two cerebrosides. The most abundant component found in the LF was sterol ester, followed in order by cerebrosides, sterol, and sterol glycoside. Lytic activity was found to be lost when samples were boiled for 5 min. An examination of the lipid composition of LF before and after boiling showed changes which may be useful in studies on the mechanism of activity of this factor. The fatty acid composition of the total lipid fraction of LF was examined by gas-liquid chromatography. The major fractions were 18:1 and 16:0 in unheated LF and 16:0 in the heated LF.

Animals↗

Frequency of arylsulphatase A pseudodeficiency associated mutations in a healthy population.

Arylsulphatase A (ASA, EC 3.1.6.1) is a lysosomal enzyme that catalyses cerebroside sulphate degradation. ASA deficiency is associated with metachromatic leucodystrophy (MLD), a rare autosomal recessive disorder, which is characterised by the storage of cerebroside sulphate. Low ASA activities can be also observed in clinically healthy persons, a condition termed ASA pseudodeficiency. Two mutations responsible for the majority of pseudodeficiency alleles have been defined in the ASA gene. These are both A-->G transitions. One causes an asparagine to serine substitution (N350S). The second changes the first polyadenylation signal downstream of the stop codon (1524 + 95A-->G), which causes a severe deficiency of one ASA mRNA species. The incidence of the pseudodeficiency allele is estimated to be high in the general population and can be found in families carrying MLD associated mutations. We report a reliable stratagem for detecting the two PD associated mutations separately, which we have applied to a healthy population. Two homozygotes for the N350S and 1524 + 95A-->G mutations were detected, which gives a population frequency of 2.6%. The overall frequencies of the ASA-PD mutations were shown to be 17.5% for the N350S change and 13.0% for the 1524 + 95A-->G change, estimating each mutation separately. In addition, the frequency of both PD associated mutations occurring together on the same chromosome was found to be 12.3% in our population. The study has also allowed us to establish a new control ASA activity range, which was based on assay of blood from persons who had been shown at the DNA level not to carry ASA PD associated mutations.

Base Sequence↗

Neutral monoglycosylceramides in rat brain: occurrence, molecular expression and developmental variation.

Developmental expression of neutral monoglycosylceramides (MGCs) has been examined in rat brain from embryonic day 15 (E15) to postnatal day 30 (P30) and adulthood. To this point, glucosylceramide (GlcCer) is the only MGC that has been characterized in embryonic brain. Galactosylceramide (GalCer) appears at P1, increases with age until P25 and remains constant thereafter. The developmental occurrence of GlcCer and GalCer agrees well with their respective glucosyl- and galactosyltransferase activities. Cerebroside fatty-acid and base compositions, examined by gas chromatography, also change during development. Several alkali-labile fast-migrating cerebrosides (FMCs) with a higher thin-layer chromatography RF than GalCer/ GlcCer are expressed early at P10, increase in concentration with age (P25-P30) and are unchanged until maturity. They are derivatives of GalCer. By employing a newly developed neutral methylation procedure, we have confirmed the structure of one of the FMCs as 6-acylGalCer. A reduction in brain FMC concentrations along with GalCer in murine genetic dysmyelinating disorders (jimpy and quaking) further supports the conclusion that they are myelin constituents.

Animals↗

Biologically active glycosides from Asteroidea, 41. Isolation and structure determination of glucocerebrosides from the starfish Linckia laevigata.

A new glucocerebroside, linckiacerebroside A (1) and a known glucocerebroside S-2a-3 (2), have been isolated from the cerebroside molecular species obtained from the less polar fraction of the CHCl3/MeOH extract of the starfish Linckia laevigata, together with three pseudo homogeneous glucocerebroside, 3, 4, and 5. The structures of these cerebrosides were determined on the basis of chemical and spectroscopic evidence.

Animals↗

Ethanol-induced changes in glycolipids of Saccharomyces cerevisiae.

Total glycolipid content of Saccharomyces cerevisiae cells increased in ethanol-treated yeast cells. Sialic acid and hexosamine contents of glycolipids from ethanol-treated cells decreased, whereas those of hexoses increased. Increased sialidase activity in the presence of ethanol may be responsible for the decrease in sialic acid content of glycolipids. The saccharide moieties of glycolipids of S. cerevisiae consisted of fucose, mannose, galactose, and glucose. Ethanol treatment of yeast cells caused an increase in glucose and a decrease in galactose content of glycolipids. The changes in glucose content can be related to changes in beta-glucosidase activity under alcohol stress. The content of cerebrosides, sulfatides, and monoglucosyldiglycerides was enhanced following ethanol treatment. An increase in cerebroside as well as in sulfatide content during alcohol stress might play an important role in stabilizing the membrane both physically and structurally. Such variations in glycolipid content and composition of S. cerevisiae cells may represent an adaptive response to ethanol stress.

Cerebrosides↗

Effect of 3-acetylpyridine on the development of rat brain.

3-Acetylpyridine, an antagonistic agent of nicotinic acid, was given to suckling rats from 6 days of age. Rats were killed at 6, 9, 12, 15, 18, 21, 24, 27 and 30 days of age respectively, and their brains were analyzed for several biochemical parameters of brain growth and myelination for comparison with those of pair-fed and ad libitum control rats. Results of DNA, RNA and protein content measurements in the brain of rats which had received 3-acetylpyridine and of pair-fed control suggested a retardation of about one week in brain growth and development compared to ad libitum control with most striking differences noted at 12 days of age. At 30 days of age, rats which had received 3-acetylpyridine showed lower values in yield of myelin, content of cerebroside and specific activity of 2', 3'-cyclic nucleotide-3-phosphohydrolase, when compared with pair-fed and ad libitum controls. These results indicate that undernourishment due to restricted food intake during brain growth-spurt results in retardation of brain development to some extent. They also suggest that nicotinic acid plays an important role in myelination associated with synthesis of cerebroside which contains high levels of long-chain fatty acid.

Aging↗

Plasma galactocerebrosides in multiple sclerosis.

Plasma cerebrosides were examined in 12 cases of multiple sclerosis which were compared to normal controls and patients with cerebrovascular accidents. A screening was performed so as to select patients with levels above to normal by means of thin-layer chromatography of the lipid extract. Glucose seems to be the only hexose to be found normally in plasma cerebrosides. The presence of galactocerebrosides may be a biological test for demyelination, as their presence seems to be related to the evolutionary state of multiple sclerosis. In a severe cerebrovascular accident, galactocerebrosides were also found.

Adult↗

Further study on cerebral sphingolipids including gangliosides in two cases of juvenile amaurotic family idiocy (Spielmeyer-Vogt type) using a new analytical procedure of sphingolipids.

Sphingolipids isolated from cerebral grey and white matter of two patients with Juvenile Amaurotic Idiocy (Spielmeyer-Vogt Type) were studied. A new analytical procedure was attempted for the determination of sphingolipids, i.e., cerebroside, sulfatide, sphingomyelin and gangliosides were subjected to ozonolysis and reduced with NaBH4. Fatty alcohols thus derived from the double bond-containing long chain bases of the sphingolipids were analyzed by GLC as their TMS-derivatives using an internal standard. The new procedure was suitable for the analysis of small amounts of sphingolipids and could determine the amounts of C18 and C20 sphingosines. It was found that all individual gangliosides in both cases gave lower proportions of C20 sphingosine to the total long chain bases. Sphingomyelin in normal human grey matter contained a small but significant amount of C20 sphingosine, while the sphingomyelin of the two patient brains indicated a much lower proportion of C20 sphingosine in comparison with those of age-matched controls. Thus, this disease seemed to be related to a genetical defect in the metabolic regulation of the long chain bases of gangliosides and grey matter sphingomyelin. On the other hand, it was noted that the ganglioside pattern of grey matter in case-1 was entirely different from that of case-2. The grey matter gangliosides in case-1 were composed of 1.78% of GM2, 81.19% of GM1 and 17.02% of GD1b by the amounts of long chain bases, while the grey matter gangliosides in case-2 seemed to be similar to those of normal human brains. Also, unusual fatty acid compositions of galactosphingolipids (cerebrosides and sulfatides) were observed to a somewhat extent in the grey matter of case-1.

Adult↗

Characterization of myelin of chick sciatic nerve during development.

Myelin was isolated from the sciatic nerves of chicks of ages 18-day embryonic, 1-day, 4-day, 7-day post-hatch, and adult to study developmental changes in lipid composition of this structure. The yield of myelin increased throughout the early stages of development and the preparations were of high purity. Although the lipid content of the myelin did not change, significant changes took place in lipid composition during development. The most significant changes were a relative increase in cerebrosides, phosphatidalethanolamine and long-chain fatty acids of cerebrosides, and a relative decrease in the content of phosphatidylserine and phosphatidylethanolamine. A second fraction ("lower band") was obtained during the isolation procedure. This "lower band" was present at all developmental stages and layered consistently at the interface of 1.2 and 0.8 M sucrose on a discontinuous gradient. The quantity of this fraction did not change during development and it differed from myelin in electron microscopic appearance. Its lipid composition, which did not change, resembled that of 18-day embryonic myelin in its high phospholipid:cholesterol ratio and low galactolipid content. The enzyme, 2':3'-cyclic-nucleosidemonophosphate phosphodiesterase was found to be present in both the myelin and "lower band" fractions; however there was no enrichment of this enzyme in purified myelin.

Aging↗

Antibodies to diverse lipids in the serum of patients with clinically cured leprosy and tuberculosis.

In this study we looked for the presence of antibodies to cardiolipin, cerebrosides, and whole lipids extracted from M. leprae, M. tuberculosis and M. habana, in the serum of patients with clinically cured lepromatous leprosy (sixteen) or tuberculosis (sixteen), 8 to 12 months after arresting the corresponding multi-drug therapy (MDT). Compared to healthy controls (sixteen), both leprosy and tuberculosis ex-patients had still significant levels of antibodies to the three mycobacterial lipids but no detectable levels of antibodies to cardiolipin or cerebroside lipids. Although leprosy and tuberculosis sera recognized the homologous mycobacterial lipids in a preferential fashion, all of them, on the average, reacted more strongly with the lipids of M. habana. This observation backs up, in a certain way, the proposition of using M. habana as a prospective vaccine for leprosy and tuberculosis.

Adult↗

[A study on the chemical constituents from Polyporus ellissi].

OBJECTIVE: To study the chemical constituents of Polyporus ellissi. METHOD: Silica gel column chromatography was applied for the isolation and purification of the constituents. The structures were established by means of spectroscopic and chemical data. RESULT: Six compounds were obtained and identified as cerebroside B (I), cerebroside D (II), ergosterol peroxide (III), 9(11)-dehydroergosterol peroxide (IV), mannitol (V) and palmitate-1-glycerol (VI). CONCLUSION: Compounds (I) and (II) were isolated from the genus Polyporus for the first time.

Cerebrosides↗

[The effect of chronic alcoholic intoxication on the level and metabolism of glycolipids in the rat brain].

The chronic alcohol intoxication has been studied for its effect on the content of glycolipids in the rat brain and incorporation of [I-14C]acetate into them. It is established that administration of ethanol to animals (2 g per 1 kg of body weight daily for 7 days) rises the content of cerebrosides I in the brain tissue. The specific radioactivity of sulphatides I falls as a result of a decrease of the [I-14C]acetate into fatty acids and galactose. The specific radioactivity of sulphatides II, cerebrosides II and III falls as a result mainly of a decrease of the specific radioactivity in the galactose components.

Alcoholism↗

An adult case of adrenoleukodystrophy with features of olivo-ponto-cerebellar atrophy: II. Lipid biochemical studies.

Different portions with or without demyelination or degeneration of formalin-fixed brain tissues of a patient with adrenoleukodystrophy and a control subject were applied to analyses of lipids, particularly sphingolipids and cholesteryl ester. Demyelinated area of the white matter in the occipital lobe showed marked decrease in cerebroside and sulfatide except for sphingomyelin and, conversely an accumulation of cholesteryl ester, whereas un-demyelinated white matter in the frontal lobe showed no abnormalities in lipids. Abnormalities of lipids in degenerated lateral nuclei of the thalamus were not so remarkable as the demyelinated white matter, whereas apparently normal dorsomedial nuclei of the thalamus showed no abnormalities in lipids. With regard to the fatty acid composition of abnormal lipids in the demyelinated white matter, all sphingolipids of cerebroside, sulfatide, and sphingomyelin showed remarkable reduction of their longer chain fatty acids and, conversely a significant increment of shorter chain fatty acids. However, these fatty acids in the degenerated lateral nuclei of the thalamus were not so different from those in the undemyelinated and apparently normal areas as well as in control brain. The fatty acids of cholesteryl ester contained mainly C18:1 and C16 acids, and very long chain fatty acids, namely fatty acids with chain length more than 22 carbons, by about 22% of the total fatty acids. In view of the analytical results of the fatty acid composition of brain lipids, it was inconceivable that this ALD patient brain showed especially the accumulation of very long chain fatty acids, and that the biochemical defect in this disease was related to the abnormal oxidation of very long chain fatty acids in peroxisomes. However, the neuropathological findings of demyelination, reactive astrocytosis, and massive infiltration of foam cells well correlated with the abnormalities in myelin lipids and the accumulation of cholesteryl ester. Also, the lower values of urinary 17-ketosteroid and 17-hydroxycorticosteroid suggested that the failure of ACTH to stimulate corticoid secretion seemed to indicate the relationship between the adrenocortical insufficiency and the affected areas of the central nervous system.

Adrenoleukodystrophy↗

Long-chain bases in the sphingolipids of atherosclerotic human aorta.

Long-chain bases were prepared from human aorta sphingomyelin by a combined enzymatic hydrolysis-alkaline hydrolysis procedure and these bases were isolated by thin-layer chromatography. Aldehydes, obtained from the long-chain bases by periodate oxidation, were converted to 1,3-dioxolane derivatives. Dioxolanes were identified and quantified by gas-liquid chromatography before and after catalytic hydrogenation, and before and after separation into saturated, monoene, and diene dioxolane fractions. The monoene dioxolanes were converted to aldehydes by reductive ozonolysis with dimethyl sulfide and these aldehydes were isolated and identified as dioxolane derivatives. The double bond positions in the major diene component were established by reductive ozonolysis and permanganate-periodate oxidation. Sphingenines in the cerebroside-sulfatide and sulfatide fractions of aorta were converted to aldehydes by the reductive ozonolysis of intact sphingolipids and these aldehydes were analyzed as the dioxolanes. Human aorta sphingomyelin contained significant amounts of 4-hexadecasphingenine, 4-heptadecasphingenine, sphinganine, 4-sphingenine, and 4,x14-sphingadienine. Small amounts of hexadecasphinganine, 4-tetradecasphingenine, a sphingadienine isomer, an unknown sphinganine, and two unknown diene long-chain bases were also found in sphingomyelin. The presence of a branched-chain 4-sphingenine was tentatively established and the possible presence of a sphingenine isomer was suggested. The major sphingenines were the same in the sphingomyelin, sulfatide, and cerebroside-sulfatide fractions of human aorta.

Aged↗

Glycolipids of peripheral nerve: isolation and characterization of glycolipids from rabbit sciatic nerve.

Besides cerebreside and sulfatide four other glycolipids were isolated from rabbit sciatic nerve and analyzed by chemical and chromatographic methods. Three of the glycolipids were shown to be fatty acid esters of cerebroside; the fourth was characterized as diacyl glycerol galactoside and its alkyl ether analog. In the ester linkage mainly unsubstituted acids with chain length C(16) to C(18) were present. Both hydroxy and unsubstituted acids were found in amide linkage. They varied in chain length from C(16) to C(24) and were typical of cerebrosides. The long-chain base fraction contained sphingosine and dihydrosphingosine as the main components.

Animals↗

A simplified assay method for galactosyl ceramide beta-galactosidase.

In a previously described method for determining the activity of cerebroside galactosidase, the enzyme preparation was incubated with an emulsion of cerebroside which had been labeled in the galactose moiety. The liberated galactose was separated from the emulsion by liquid-liquid partitioning, but the presence of detergent necessitated the use of careful agitation and a backwash in order to reduce the contamination of the aqueous layer with excess emulsified substrate. This problem is eliminated by adding a large amount of lipid to reduce the emulsifying power of the detergent. It may be that other lipid hydrolase assays, based on the same principle, would benefit by this approach. Some additional improvements in the assay system are described.

Bile Acids and Salts↗