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PubMed1984

Effect of C 18:3 (n-3) dietary supplementation on the fatty acid composition of the rat brain.

Weanling rats have been fed different diets containing hydrogenated coconut oil or a mixture of grapestone + linseed oil as dietary fats. Groups of 12 animals have been sacrificed at weanling or after 60 or 120 days of this dietary treatment. Body and brain weight, brain total lipids, phospholipids, cholesterol, cerebroside, fatty acid content and fatty acid composition have been evaluated. The diet rich in alphalinolenic acid (C 18:3 n-3) induced a significant increase of the n-3 polyunsaturated fatty acids (C 22:5 and C 22:6, n-3) in the brain, with respect to the other experimental conditions.

Animals↗

PubMed1981

Hydrophobic compounds interfere in radioimmunoassay for basic protein in myelin.

Hydrophobic compounds influenced the accuracy of the radioimmunoassay for myelin basic protein when lipids (stearic acid, phosphatidylcholine, cholesterol, cerebroside, sulfatide, or GM1 ganglioside) or proteolipids (white-matter proteolipid apoprotein, kidney proteolipid apoproteins, or heart proteolipid apoproteins) were added to a known amount of basic protein and the samples assayed. All of these interfere with the assay, but the direction of the error depends on the quantity added: low concentrations of lipid decrease apparent basic protein, high concentrations enhance it. Obviously, results of basic-protein assays must be interpreted carefully.

Animals↗

PubMed1984

Role of myelin antigens in murine relapsing experimental allergic encephalomyelitis.

Delayed, relapsing experimental allergic encephalomyelitis (DR-EAE) has been shown to be a good model of multiple sclerosis. This disorder has been produced in mice by immunization with whole central nervous system (CNS) tissue and adjuvants. The ability of various myelin components to produce DR-EAE was studied. Guinea pig myelin basic protein was able to induce DR-EAE that was clinically and pathologically identical to disease produced by whole CNS. Both acute inflammatory and chronic demyelinating lesions were seen in the CNS. Allogeneic mouse myelin basic protein either alone or in combination with cerebrosides and/or gangliosides was not able to produce DR-EAE.

Animals↗

PubMed1984

Long-term metabolism of [U-14C]glucose in the whole rat brain.

The experiment was performed on rats to which a single injection of [U-14C]glucose had been administered. Results were observed from the 7th to the 281st day following contamination. At 280 days only the lipids in the brain contained radioactivity, the highest degree of specific activity being found in the cerebrosides.

Animals↗

PubMed1978

Metabolic properties of maintained oligodendroglia purified from brain.

Oligodendroglia isolated in bulk from dissected white matter of lamb or calf brain can now be maintained in culture for several days. The cells remain intact and viable during this time. The in vitro incorporation of various radiolabeled precursors into lipids or proteins was examined, and its was found that oligodendroglia synthesize the lipids which are predominant in brain, and especially in myelin. They actively synthesize cholesterol, cerebrosides, sulfatides, and all of the phospholipids, especially phosphatidylcholine. In addition, they produce a variety of radiolabeled proteins, with those of high molecular weight exhibiting the most radioactivity. Thus the cells are metabolically very active in vitro and can be used as a model system to study turnover of lipids in the cell, as well as the synthesis of myelin-specific components.

Animals↗

PubMed1982

Cerebrospinal fluid markers in African Burkitt's lymphoma with central nervous system involvement.

Several cerebrospinal fluid markers were found to be elevated in Burkitt's lymphoma patients with central nervous system (CNS) involvement. Antibody levels to the virus capsid antigen of the Epstein-Barr virus and to the brain cell antigens myelin and cerebroside were elevated during active CNS disease. Immune complexes were present in levels above 100 micrograms/ml in most patients with CNS involvement but tended to be low or negative in patients without CNS disease. Oligoclonal IgG bands were present in 12 of 13 patients with CNS disease and in only 3 of 26 patients with no clinical evidence of disease. None of these markers were present in 6 other tumor patients without CNS disease. The presence of these markers in 12 of 13 patients in whom CNS disease was involved suggests that these markers may be useful in determining the status of the tumor with regard to involvement of the CNS.

Antibodies↗

PubMedDate not supplied

[Guinea pig brain and spinal cord glycolipids in tricresyl phosphate poisoning].

Experimental neuroparalytic form of chronic intoxication with tricresylphosphate (TCP was induced in adult guinea pigs by means of single intracutaneous administration of TCP (industrial mixture containing 37% of ortho-isomer) at a dose of 2.0-2.2 ml/kg of body weight. Moderate and severe forms of the impairment, accompanied by paresis and paralysis of hind extremities was developed in 66.1% of the treated animals. Content of galactolipids (cerebrosides + sulphatides) and gangliosides was determined in brain stem, lumbar and sacral parts of spinal cord of intact and impaired animals. Content of galactolipids was distinctly decreased in spinal cord (by 22.9%) and in brain stem (9.0%). Total gangliosides were decreased by 19.1% in spinal cord, but its content was altered in brain stem. These alterations appear to reflect destructive processes not only in myelin membranes but also in structure of neurones.

Animals↗

PubMedDate not supplied

[Effect of experimental hyperphenylalaninemia on the metabolism of gluco- and galactocerebrosides and sulfatides].

Alterations in content of gluco- and galactocerebrosides were studied in brain of rats of various age. Specific radioactivity of glucocerebrosides exceeded distinctly that of galactocerebrosides. When chronic hyperphenylalaninemia was produced in adult rats, the specific radioactivity of brain cerebrosides and sulfatides decreased significantly. Content of these glycolipid fractions decreased only slightly in liver and spleen tissues.

Animals↗

PubMed1976

Enhancement of sulphatide metabolism in the hypertrophied kidney of C3H/He mouse with reference to [Na+, K+]-dependent ATPase.

Sulphatide (cerebroside sulphate) metabolism of C3H/He mouse kidney was investigated in the course of compensatory renal hypertrophy in association with the change of [Na+,K+]-dependent ATPase, arylsulfatase A and beta-galactosidase activity. A remarkable increase in 35S incorporation into kidney sulphatide was observed 24 hours and especially 7 days after unilateral nephrectomy. In contrast, no significant alteration of 32P incorporation into major phospholipids such as phosphatidylcholine, phosphatidylethanolamine and sphingomyelin was demonstrated in the compensatory hypertrophied mouse kidney. [Na+, K+]-dependent ATPase increased to 126% of control in the remaining kidneys on 7 days after operation. Specific increase in 35S specific activity of kidney sulphatide suggests its possible link with the process of active ion transport through membrane-bound [Na+,K+]-dependent ATPase. Arylsulphatase A activity increased to 151% of control on days, while little change was observed in beta-galactosidase activity. These results suggest a sole concern of a turnover of sulphate moiety of sulphatide molecule in the elevated metabolism.

Adenosine Triphosphatases↗

PubMed1978

[Blood glycolipids of patients with cerebral circulatory disorders].

The paper is concerned with a study of the glycolipid content in the blood of 70 patients with acute disorders of brain circulation and in 14 patients with the initial signs of insufficiency of circulation. It was established that in the plasma and blood cells in patients with ischemic and hemorrhagic strokes the amount of gangliosides and cerebrosides were considerably higher than in normals (35 cases). In patients with transient disorders of brain circulation and initial signs of insufficiency of brain circulation the content of glycolipids is increased to a lesser degree. A conclusion has been made that there is a possible involvement of oligosaccharidceramides (mono- and asyalgangliosides, galactocerebrosides) in the occurrence of disorders in cerebral hemodynamics.

Acute Disease↗

PubMed1995

Successful transduction of oligodendrocytes and restoration of arylsulfatase A deficiency in metachromatic leukodystrophy fibroblasts using an adenovirus vector.

Metachromatic leukodystrophy (MLD) is an inherited metabolic disease which is characterized by deficient activity of arylsulfatase A (ASA). This deficiency causes progressive accumulation of cerebroside sulfate (CS) in oligodendrocytes (OLs) in the brain resulting in dysmyelination. We are developing approaches for treating MLD based on direct delivery of ASA genes to brain. In this paper, we report a recombinant adenovirus (Adex1SRLacZL) that efficiently transduces OLs. Moreover, transduction and expression of the human transgene was efficient in primary fibroblasts from MLD patients exposed to similar recombinant adenovirus carrying the ASA gene (Adex1SRASA) In addition, the expressed ASA protein was correctly targeted to lysosomes and was immunoreactive to anti-ASA antibody. The enzymatic activity of ASA in MLD patient cells is corrected from the deficient state to 2.3 to 5.0 times the activity of normal control cells. These results demonstrate the correction of ASA deficiency by a recombinant adenovirus that is potentially useful for transferring the gene to brain.

Adenoviridae↗

PubMed1975

Purification and properties of arylsulfatase. A from human urine.

Arylsulfatase A (cerebroside sulfate sulfohydrolase) was purified 3500-fold at a 7% yield from human urine. A crude urinary protein concentrate was prepared by treating pooled urine with ammonium sulfate and subsequently drying the precipitate with acetone. The powder thus obtained was extracted with buffer and was subjected to chromatographic and electrophoretic procedures as follows: (a) ammonium sulfate reverse gradient solubilization chromatography; (b) DEAE-cellulose chromatography; (c) Sephadex G-200 gel filtration; (d) preparative polyacrylamide gel electrophoresis; (e) SP-Sephadex chromatography; and (f) antialbumin-Sepharose chromatography. The enzyme was judged to be essentially homogeneous by: (a) a single band on polyacrylamide gel electrophoresis at two pH values; (b) formation of a single precipitin line on immunodiffusion against its antiserum: (c) complete freedom from albumin, the major contaminating protein; and (d) a single band on sodium dodecyl sulfate gel electrophoresis.

Catechols↗

PubMed1976

[Study of the passage of stearic acid through blood-brain barrier and its incorporation in cerebral membranes (especially in myelin)].

Subcutaneously injected stearic acid is uptaken by brain and is further incorporated into membrane lipids (especially myelin). The uptake increases regularly up to 20 hrs. in total membranes as in myelin. In total membranes, there is a decrease between 20 and 24 hrs. followed by a recovery of the previous maximal activity. Moreover, the myelin activity increases up to 3 days, so far. Cerebrosides, isolated from both types of preparations, present an activity regularly increasing; but free fatty acids have a stable specific activity and a decreasing relative activity. The injected labelled stearic acid is directly incorporated into membrane lipids or is metabolized inside brain in longer chains (thus providing arachidic behenic and lignoceric acids) or in acetate units (utilized for synthesis of medium chain fatty acids such as palmitic acid).

Animals↗

PubMed1994

Genetics of metachromatic leukodystrophy.

Metachromatic leukodystrophy (MLD) is a lysosomal storage disease caused by the deficiency of arylsulfatase A (ASA). The mode of inheritance is autosomal recessive. The disease occurs panethnically and its frequency is 1 in 40000. The deficiency of the enzyme causes the accumulation of its substrate cerebroside sulfate. Since this sphingolipid is mainly found in the myelin membranes the disease primarily affects the oligodendrocytes. Patients suffer from a progressive demyelination and die due to a variety of neurologic symptoms. Clinically the disease is heterogeneous. Depending on the age of onset a late infantile, juvenile and adult form can be distinguished. We have cloned the cDNA and gene of arylsulfatase A. Several disease causing mutations have been identified and a simple genotype phenotype correlation has been revealed. Currently we try to develop a mouse model of MLD via homologous recombination in embryonic stem cells. The model will allow to elucidate the pathogenesis of the disease and to test possible therapeutic approaches.

Adult↗

PubMed1996

Coagulation abnormalities in patients with Gaucher's disease: effect of therapy.

Various coagulation defects have been associated with Gaucher's disease, including factor IX deficiency and acquired von Willebrand's disease (VWD). We performed repeated coagulation assays in 9 patients with Gaucher's disease over a period of 2 years. The prothrombin time (PT) and fibrinogen levels were normal in 8 of 9 patients, while the partial thromboplastin time (PTT) was abnormal in 5 of 9; all mixing PTT tests showed correction. Factor IX was normal repeatedly in the 7 of 7 patients tested. In contrast, factor XI was decreased in 3 of 9 patients assayed. Anticardiolipin (ACL) IgM was normal in all patients. ACL IgG was highly variable; levels were abnormal at least once in 6 of 8 patients, but were also normal at least once in 7 of 8 patients. Factor VIII was also quite variable: levels were decreased at least once in 4 of 9 patients, and normal at least once in 8 of 9 patients. Von Willebrand factor antigen (VWF Ag) studies were normal in 7 of 8 patients, but VWF activity was decreased at least once in 4 of 8 patients. In some patients, these problems could be overcome by specimen dilution. In ony 1 patient was VWF Ag decreased; this patient had a factor VIIIC level of 13% , and VWF activity of 18.7%. Coagulation assays performed before and after alglucerase administration failed to demonstrate any significant improvement in these assays, and neither was there a consistent improvement over the duration of therapy. We suggest that previously reported decreases in factor IX and VWF may be secondary to the interfering presence of increased cerebroside levels. Caution must be used in the interpretation of clotting assays in the patient with Gaucher's disease.

Antibodies, Anticardiolipin↗

PubMed1996

Sulfatides as a predictive factor of lymph node metastasis in patients with colorectal adenocarcinoma.

BACKGROUND: Sulfatide is one of the acidic glycolipids that are components of the cellular membrane. It has been reported that sulfatide plays some important roles in cell functions, such as cell adhesion. The aim of this study was to investigate the relationship between sulfatide and the malignant potential of colorectal carcinoma. METHODS: Glycolipids were extracted from 22 primary colorectal cancer tumors and 6 adjacent normal mucosa using the Folch method. Qualitative analysis of the sulfatide contents was performed using thin-layer chromatography (TLC) and TLC immunostaining. Quantitative analysis was performed by densitometric scanning. RESULTS: Two bands of sulfatide were observed by TLC immunostaining and were designated as cerebroside sulfated ester (CSE)-A and CSE-B. Levels of CSE-A were found to be significantly decreased whereas those of CSE-B were significantly increased in cancerous tissue when compared with normal tissue (P<0.05). The CSE ratios (CSE-B/[CSE-A + CSE-B]) in the 15 tumors showing lymph node metastasisi were higher than in the 7 tumors without lymph node metastasis (P<0.05). The CSE ratios in 9 Dukes Stage C tumors and 6 Dukes Stage D tumors were higher than those in 7 Dukes Stage A tumors (P<0.02, and P<0.05, respectively). CONCLUSIONS: These data support the conclusion that changes in sulfatide composition may play an important role in lymph node metastasis of colorectal adenocarcinoma.

Aged↗

PubMed1996

Experimental autoimmune encephalomyelitis: antigen-induced inhibition of biochemical and immunohistological alterations.

A comprehensive biochemical, immunological, and histological study was undertaken during suppression of experimental autoimmune encephalomyelitis (EAE) induced by antigen-specific inhibition of the immune response. Pretreatment of Wistar rats by intraperitoneal administration of low doses of saline-soluble bovine myelin or myelin basic protein (MBP) but not with ovalbumin suppresses the appearance of the clinical symptoms of EAE induced by sensitization with bovine myelin in complete Freund's adjuvant. Analysis of the central nervous system (CNS) of animals pretreated with MBP or whole myelin shows inhibition of the diminution of MBP and 2',3'-cyclic nucleotide 3'-phosphohydrolase (CNPase) activity observed in the EAE animals or in rats pretreated with ovalbumin. With respect to the CNS lipid content, these suppressive treatments abolish the increase in esterified cholesterol and partially revert the diminution in the content of cerebrosides and total cholesterol characteristic of the acute stage of the disease. Concomitantly, meningeal and parenchymal infiltration with mononuclear cells and deposits of immunoglobulins in the infiltrated regions as well as in spinal cord motor neurons were reduced. Analysis of the humoral response to myelin antigens shows that all EAE as well as treated animals developed antibodies to MBP and other myelin proteins. However, a higher incidence and level of these antibodies was observed in nontreated EAE animals and MBP- and ovalbumin-treated rats, while rats treated with total bovine myelin showed a highly reduced humoral response. The present results indicate that intraperitoneal treatment with soluble forms of myelin antigens, concomitant with the suppression of the clinical symptoms of the disease, markedly reduces the biochemical and histological alterations occurring in EAE animals and produces changes in the autoimmune humoral response.

Allergens↗

PubMed1998

Transport of proteolipid protein to the plasma membrane does not depend on glycosphingolipid cotransport in oligodendrocyte cultures.

The possibility that transport of proteolipid protein (PLP) from its site of synthesis to the plasma membrane is dependent on cotransport with (sulfo)galacto-cerebrosides was investigated in primary cultured oligodendrocytes and Chinese hamster ovary (CHO) cells expressing PLP. Sulfation was inhibited by growing oligodendrocytes in the presence of a competitive inhibitor of this process, sodium chlorate. Under these circumstances, sulfatide synthesis was inhibited by 85%. Nevertheless, PLP was still delivered to the plasma membrane in quantitative amounts. Furthermore, when PLP was expressed in CHO cells, which normally synthesize very low amounts of galactosyl ceramide (GalCer) and no sulfatide, PLP was transported to the plasma membrane. Moreover, in CHO cells coexpressing PLP and ceramide galactosyl transferase, PLP cell surface labeling was unaltered. Noting that it has been demonstrated that proteins destined for the apical surface of epithelial cells colocalize with glycolipid-enriched microdomains, we isolated detergent-insoluble membrane complexes from cultured oligodendrocytes. We found, however, that most of the PLP is present in the detergent-soluble fraction and, furthermore, that PLP could not be chased into or out of the insoluble fraction. Taken together, these data make it very likely that in oligodendrocytes PLP transport takes place irrespective of the presence of glycosphingolipids GalCer and sulfatide.

Animals↗