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PubMed1986

Abnormalities of cerebral lipids in GM1-gangliosidoses, infantile, juvenile, and chronic type.

Cerebral lipids of patients with GM1-gangliosidoses, infantile, juvenile, and chronic type which are caused by deficiency of beta-galactosidase, were examined and compared to each other. The infantile type demonstrated abnormal accumulation of GM1 and asialo-GM1 in contrast with marked decrease in such major cerebral lipids as cholesterol, phospholipids, cerebroside, and sulfatide. It was also noted that significant amounts of such unusual lipids as free fatty acids, GlcCer, LacCer, GbOse3Cer, and GbOse-4Cer plus nLcOse4Cer were found in the brain. These findings pointed out that this infantile type might accompany a severe cerebral dysgenesis with poor myelination. The juvenile type also showed marked increase in GM1 and asialo-GM1, but the decrease in cholesterol, phospholipids, cerebroside, and sulfatide was not so much as the infantile type. These findings along with the occurrence of cholesterol ester suggested that the brain caused progressive demyelination after the immature myelin appeared. An autopsized brain tissue of a male patient who was eventually diagnosed as a case of GM1-gangliosidosis chronic type after his death, showed some accumulation of GM1 and asialo-GM1 particularly in the caudate nucleus and putamen, whereas it showed moderate amounts of GM1 in apparently normal gray and white matters. It seemed that there are no abnormal cerebral lipids except for gangliosides and some neutral glycosphingolipids in the chronic type.

Adult↗

PubMed1988

Warfarin administration reduces synthesis of sulfatides and other sphingolipids in mouse brain.

The modulation of phosphosphingolipid synthesis by vitamin K depletion has been observed in the vitamin K-dependent microorganism, Bacteriodes levii. When cultured briefly without the vitamin, a reduction occurred in the activity of the first enzyme of the sphingolipid pathway, 3-ketodihydrosphingosine synthase. In this report, 16-day-old mice were treated with the vitamin K antagonist, warfarin. Brain microsomes from these animals showed a 19% reduction in synthase activity. Mice treated with warfarin for 2 weeks showed a major reduction in sulfatide level (42%), with a lesser degree or no reduction in levels of gangliosides and cerebrosides. In further experiments, mice were treated with warfarin for 2 weeks and a group was then injected with vitamin K1 (aquamephyton) for 3 days. Enzyme activity returned to a normal level within 2-3 days. Sulfatide levels had increased 33% in the vitamin K-injected group and ganglioside levels also increased, where levels of cerebrosides and sphingomyelin declined. Sulfatide synthesis determined by [35S] sulfate incorporation, showed a 52% increase in incorporation following administration of vitamin K for 3 days. These results suggest a role for vitamin K in the biosynthesis of sulfatides and other sphingolipids in brain. This putative role could be by post-translational protein modification analogous to the role of vitamin K in other systems.

Animals↗

PubMed1979

Attempt at enzyme replacement in Gaucher disease by renal transplantation.

In Gaucher disease there is a deficiency of the lysosomal enzyme, cerebroside-beta-glucosidase, as a result of which cerebroside (glucosylcereamide) accumulates in various organs. In northern Sweden 22 patients with a juvenile form of this disease have been identified. In one such patient, a girl of 10 years, we have attempted enzyme replacement by renal transplantation. After this operation the hepatic glucocerebroside content fell significantly. In another child afflicted with Gaucher disease in whom splenectomy was performed for severe splenomegaly and hypersplenism there was a progressive increase in the level of this lipid. These findings suggest that enzyme replacement was achieved by transplantation of a normal organ.

Child↗

PubMed1971

Lipid composition of rat brain myelin in triethyl tin-induced edema.

Chronic triethyl tin intoxication was induced in young adult rats by oral feeding of triethyl tin sulfate. Progressively severe brain edema developed during the 3-month experimental period. The yield of myelin from the brains of the experimental animals decreased to almost half normal per brain, but the isolated myelin appeared morphologically normal. The analysis of whole brain showed corresponding decreases in proteolipid protein and total lipid, particularly galactolipids. The proportions of the major constituents of isolated myelin (chloroform-methanol-insoluble residue, proteolipid protein, and total lipid) were unchanged despite the low yield. However, the proportion of cholesterol increased from 16 to 21% dry weight, and that of total galactolipid decreased from 21 to 15%, as the yield of myelin decreased. This decrease of total galactolipid was mainly due to the decrease in cerebroside. Total phospholipid remained constant initially but showed a slight decrease toward the end of the experiment, due mostly to decreased ethanolamine phospholipid. There was no preferential loss or preservation of phosphatidalethanolamine. The fatty acid composition of sulfatide showed statistically significant shifts to less long-chain fatty acids and less monoenoic acids, but cerebroside and sphingomyelin did not show significant changes in the fatty acid composition. There was no increase in esterified cholesterol. These findings generally support our hypothesis of nonspecific chemical abnormalities of the myelin sheath undergoing secondary degeneration. In an acute experiment, a single intraperitoneal injection of triethyl tin sulfate produced acute and transient brain edema. There were slight decreases in the yield of myelin, but no detectable changes in the chemical composition.

Administration, Oral↗

PubMed1982

Lipid class analysis of the central nervous system of myelin-deficient Wistar rats.

Brains and spinal cords of myelin-deficient (md) and littermate control rats were analyzed serially for myelin lipids during the period from 13 to 32 days of age. The glycolipids of md rat brains were severely reduced and remained so during the period of study; brain cholesterol and phospholipids increased moderately but never reached the values for control brains. Deficiency of all three lipid classes was marked in the spinal cord and did not change with age. Among the glycolipids of md rats, deficiency was more severe in cerebrosides than sulfatides. The pronounced reduction of cerebrosides in the early stages of myelination suggests that abnormal synthesis of these glycolipids may be the most important biochemical anomaly responsible for myelin deficiency.--Csiza, C.K. Lipid class analysis of the central nervous system of myelin-deficient Wistar rats.

Aging↗

PubMed1982

[Regulation of the superprecipitation of an actomyosin-like protein from the brain by glycolipids].

The optimal conditions (ionic strength, pH, Mg2+ and ATP concentrations) for nephelometric determination of the superprecipitation reaction of the actomyosin-like protein (AMLP) from ox brain cortex are described. At low ionic strength (0.1 M KCl, 200 micrograms of protein, pH 6.8, 37 degrees) MgATP (1 mM) first causes a fast (1--2 min) dissociation of AMLP with a decrease in the optical density (A620) at 620 nm and then a slow (15--20 min) true superprecipitation associated with a substantial rise of A620. EGTA (0.1 mM) moderately, i. e. by 20%, inhibits the reaction. The effects of glycolipids (total gangliosides and cerebrosides from ox brain) and phosphatidyl choline from chicken egg yolk on aggregation (without Mg-ATP), dissociation and superprecipitation of AMLP were examined. It was found that gangliosides (40 micrograms) and cerebrosides (2--80 micrograms) cause aggregation of AMLP; phosphatidylcholine (4--160 micrograms) has no effect on protein aggregation. Gangliosides within the concentration range of 0.8--4.0 micrograms do not cause AMLP aggregation but strongly inhibit superprecipitation of the protein, whereas phosphatidylcholine (40--80 micrograms) has practically no effect on the reaction intensity. A hypothesis on regulation by glycolipids on contractile proteins from brain presynaptic membranes under varying rest--excitation conditions is postulated.

Actomyosin↗

PubMed1981

Interaction of C-reactive protein with artificial phosphatidylcholine bilayers and complement.

We have examined the interaction of C-reactive protein (CRP) with model membranes and complement. Binding of CRP to multilamellar liposomes or unilamellar vesicles of egg-phosphatidylcholine required the presence of lysophosphatide in the bilayer. The binding was Ca++-dependent, could be inhibited by phosphocholine, and resulted in activation of the classical complement pathway. A weak interaction between CRP and agarose was observed, which was also CA++-dependent and could be inhibited by phosphocholine and galactose. In addition, incorporation of galactocyl cerebroside in phosphatidylcholine:lysophosphatidylcholine liposomes enhanced the binding of CRP. Binding constants of 1.9 X 10(-5) M and 7.1 X 10(-5) M were calculated for liposomes containing and lacking the glycolipid, respectively. Furthermore, CRP bound to galactocyl cerebroside-containing liposomes bound approximately twice as much C1q as the same amount of CRP bound to liposomes lacking the glycolipid. We conclude that: 1) An alteration of the normal organization of phosphatidylcholine bilayers is necessary for binding of CRP. 2) The presence of galactosyl residues on the surface of the bilayer enhances the binding of CRP, perhaps through interaction with a putative secondary binding site on the protein.

Animals↗

PubMed1995

[Autoantibodies to ganglioside GM3 and serotonin in blood serum in atherosclerosis].

Using ELISA method, the sera from 17 patients with atherosclerosis and 13 normal controls were examined for ganglioside- and serotonin-reactive antibodies. Gangliosides from human liver (GM31) and human aorta (GM3a) as well as GM2, GM1, GT1b, human brain cerebrosides and the BSA-serotonin conjugate (5-HT) were used as antigens. A group of patients showed statistically significant higher levels of anti-GM31 (82%) and anti-5-HT (71%) as compared to the control group. Taking into account the identical fatty acid composition of GM3 from human liver and platelets, one can assume that antibodies are produced against blood clot gangliosides in atherosclerotic patients' sera. This conclusion is supported by a high correlation (r = 0.06, p < 0.01) between the level of antibodies to 5-HT and GM31 in the sera of all patients. The sera of three patients with the highest content of antibodies to GM31 were shown to contain antibodies to GM3a and GT1b which were absent in control sera. No reaction with brain cerebrosides or GM1 and GM2 was detected in the sera of the examined persons. The differences in sera reactions with GM31 and GM3a can be explained as resulting from differences in the chain length of fatty acid residues of the ceramide moiety of gangliosides. The data obtained confirm the fact that antibodies to aorta gangliosides appear in the sera of atherosclerotic patients. Thus, the formation of atherosclerotic plaques leading to platelet activation, clot formation and ganglioside accumulation in aortic cells increase the levels of anti-ganglioside GM3 antibodies.

Aorta↗

PubMed1993

Thrombospondin related anonymous protein (TRAP) of Plasmodium falciparum in parasite-host cell interactions.

Thrombospondin related anonymous protein (TRAP) of Plasmodium falciparum is characterized by the presence of an amino acid motif based on the sequence Trp-Ser-Pro-Cys-Ser-Val-Thr-Cys-Gly (WSPCSVTCG) that is found in a growing family of proteins. The sequence WSPCSVTCG is considered to confer sulpho-galactosyl-cerebroside (sulphatide) binding properties to antistasin, TSP, CS protein and properdin. The observation that TRAP is localized both on the micronemes and on the surface of P. falciparum sporozoites would suggest a role played by TRAP, and its putative sulphated glycoconjugates binding motif, in the recognition and/or entry of hepatocytes by the sporozoite. Our results indicated that TRAP constructs, expressed in E. coli, bind to sulpho-galactosyl-cerebrosides (sulphatides) and to the surface of HepG2 cells using the conserved amino acid motif WSPCSVTCG. Antisera raised against TRAP constructs inhibited sporozoite invasion of HepG2 cells thus suggesting, thus, that TRAP may be one of the parasite-encoded molecules implicated in the sporozoite invasion of hepatocytes. Moreover, the possibility that TRAP antibodies may be relevant in malaria immunity is supported by the results obtained in a prospective study conducted in a malaria endemic area. In adolescents, the presence of TRAP antibodies, before malaria transmission, correlated positively with the control of parasite density.

Adolescent↗

PubMed1993

Less polar glycolipids in Alaskan pollack brain: isolation and characterization of acyl galactosyl diacylglycerol, acyl galactosyl ceramide, and acyl glucosyl ceramide.

We recently reported that glycolipid compositions of gadoid fish nerve tissues are unique in the abundance of 1) galactosyl diacylglycerol and its sulfate ester; 2) glucosyl ceramide; and 3) fatty acid ester of cerebroside (J. Lipid Res. 1992. 33: 1351-1359). The present paper reports the characterization of less polar glycolipids isolated from Alaskan pollack brain. Of twelve glycolipids purified by column chromatography, four were of the galactosyl diacylglycerol type. Chemical analysis, infrared spectrometry, and a permethylation study followed by gas chromatography-mass spectrometry revealed that they were 1,2-di-O-acyl-3-beta (6-O-acyl-D-galactopyranosyl)-sn-glycerol and 1,2-di-O-acyl-3-beta(2'-O-acyl-D-galactopyranosyl)-sn-glycerol, and mixtures of these two isomers, with slightly different fatty acid compositions, respectively. The other eight less polar glycolipids were pure forms or mixtures of isomeric forms of cerebroside fatty acid esters in which the substituted position of the acyl group on the hexose moiety varied. The permethylation study revealed that they were 6-O-acyl-beta-D-galactopyranosyl, 6-O-acyl-beta-D-glucopyranosyl, 2-O-acyl-galactosyl, 2-O-acyl-glucosyl, 3- or 4-O-acyl-galactosyl, and 3-O-acyl-glucosyl ceramides, the 6-O- and 2-O-acyl isomers being predominant. This is the first report of the natural occurrence of 1) acyl galactosyl diacylglycerol in animal tissues and 2) acyl glucosyl ceramide in nerve tissues.

Animals↗

PubMed1996

Thermodynamics of water-lipid interaction in presence and absence of salts.

Extents of hydration of different lipids such as cholesterol, lecithin, lysolecithin, gangliosides, hydroxy cerebrosides, sphingomyelin, Dalda (hydrogenated fat), coconut oil and sunflower oil in the presence and absence of some inorganic electrolytes, sucrose and urea have been studied using the isopiestic vapour pressure technique. Except triglycerides, the shape of the water vapour adsorption isotherms for other lipids are in agreement with type II or type III BET isotherms respectively. The overall shape of each curve for triglycerides does not agree with any of the shapes of five types of BET isotherms. At water activity (1) approaching unity, maximum number of moles of water bound (delta n1zero) per mole of cholesterol are 1.6 and 2.6 at 28 degrees C and 37 degrees C, respectively. Values of delta n1zero at 23 degrees C stand in the following order: Ganglioside > lysolecithin > sphingomyelin > hydroxy cerebrosides. For triglycerides n1 increases with increase of a1 and reaches maximum in between 0.95 to 0.98 beyond which it sharply decreases to zero as a1 approaches unity. Standard free energy changes (delta Gzero) and enthalpy changes (delta Hzero) for cholesterol and lysolecithin have been evaluated from the experimental data. The excess hydration of cholesterol in the presence of several inorganic salts, urea and sucrose have been estimated from isopiestic experiments and free energies of excess hydration have been evaluated.

Lipids↗

PubMedDate not supplied

[Brain glycolipids in rats after disulfiram administration].

The effect of disulfiram (teturam, antabus) (200 mg/kg, intragastrically) on the rat brain glycolipid structure was studied. It was found that disulfiram increased the contents of cerebrosides I and III in nervous tissue. The administration of alcohol (1 g/kg, 20% solution, intraperitoneally), with disulfiram being simultaneously administered, was accompanied by increased levels of brain cerebrosides I and II.

Alcohol Deterrents↗

PubMed1982

Presymptomatic diagnosis: metachromatic leukodystrophy or pseudo arylsulphatase A deficiency?

Diagnosis of metachromatic leukodystrophy (MLD) was established in the proband at age 27 months. An examination of the family arylsulphatase A profile revealed that the father and younger sibling, age 2 months, had very low enzyme activities like the proband. The father, in all likelihood, had the pseudo arylsulphatase A deficiency trait, but the sibling could be either pseudodeficient or affected with MLD. The fibroblast cerebroside sulphate loading test confirmed that the father had pseudo arylsulphatase A deficiency. The test also indicated that the sibling was affected with MLD. This was confirmed by clinical evidence of neurological degeneration by 18 months.

Cerebroside-Sulfatase↗

PubMed1980

Chemical characterization and substrate specificity of rabbit liver aryl sulfatase A.

Rabbit liver aryl sulfatase A (aryl-sulfate sulfohydrolase, EC 3.1.6.1) is a glycoprotein containing 4.6% carbohydrate in the form of 25 residues of mannose, seven residues of N-acetylglucosamine, and three residues of sialic acid per enzyme monomer of molecular weight 140 000. Each monomer consists of two equivalent polypeptide chains. The protein has a relatively high content of proline, glycine and leucine, and the amino acid composition of rabbit liver aryl sulfatase A is similar to that of other known liver sulfatases. Rabbit liver aryl sulfatase A catalyzes the hydrolysis of a wide variety of sulfate esters, although it appears possible that cerebroside sulfate is a physiological substrate for the enzyme because the Km is very low (0.06 mM). The turnover rate for hydrolysis of nitrocatechol sulfate or related synthetic substrates is much higher than the rate with most naturally occurring sulfate esters such as cereroside sulfate, steroid sulfates, L-tyrosine sulfate or glucose 6-sulfate. However, the turnover rate with ascorbate 2-sulfate is comparable to the rates measured using most synthetic substrates. These results are discussed in relationship to several previously described sulfatase enzymes which were claimed to have unique specificities.

Amino Acids↗

PubMed2004

Sphingolipidoses in Turkey.

During the last 5 years 2057 children under the age of 5 with various neurologic symptoms with the suspected diagnosis of lysosomal storage diseases were referred to our hospital from different universities and state hospitals. We were able to separate sphingolipidoses by lysosomal enzyme screening. A total of 300 patients (15%) with sphingolipidoses were diagnosed; there were deficiencies of arylsulfatase A [metachromatic leukodystrophy (MLD)] in 93 (31%), hexosaminidase [Sandhoff disease (SHD)] in 62 (20.7%), hexosaminidase A [Tay-Sachs disease (TSD)] in 15 (5%), beta-galactosidase (GM1 gangliosidosis) in 35 (11.7%), alpha-galactosidase (Fabry disease) in one (0.3%) cerebroside beta-galactosidase (Krabbe disease) in 65 (21.7%) and glucosylceramidase (Gaucher disease) in 29 (9.6%). SHD (20.7%), MLD (31%) and Krabbe disease (21.7%) were common. Prenatal enzymatic diagnosis was made in 70 at risk pregnancies, 64 for TSD and SHD, three for MLD and three for GM1 gangliosidosis by using chorionic villus biopsy in 54, cord blood samples in 12 and cultured amniotic fluid cells in four. Seventeen fetuses were found to be affected. We have calculated the relative frequency and minimum incidence of sphingolipidoses in Turkey. The combined incidence of sphingolipidoses is 4.615 per 100,000 live births. The calculated incidences are 1.43, 0.95, 1, 0.23, 0.54, 0.45, 0.015 per 100,000 live births for MLD, SHD, Krabbe, Gaucher, TSD, GM1 gangliosidosis and Fabry diseases, respectively. The real incidence, which covers all subtypes of this group of diseases, should be greater than this number. The results suggested that, as a group, sphingolipidoses are relatively common and represent an important health problem in Turkey and some rare autosomal recessive diseases of Turkey are due to 'founder effect' created by consanguineous marriages.

Cerebroside-Sulfatase↗

PubMed1985

Pseudoarylsulfatase-A deficiency in the neurologically impaired patient.

The demonstration of low arylsulfatase-A (ASA) activity in leucocytes or fibroblasts is used often to establish the diagnosis of metachromatic leucodystrophy (MLD). However, low ASA activity is observed also in pseudo-ASA deficiency which may be as common as MLD. We report two patients with pseudo ASA deficiency who had abnormal neurological findings consistent with atypical MLD. Because the measurement of ASA activity is neither a sensitive nor specific method with which to establish a diagnosis of MLD, this diagnosis should be confirmed by nerve biopsy, measurement of urinary sulfatide or a cerebroside sulfate loading test, using cultured fibroblasts.

Adult↗

PubMed1991

Restoration of arylsulphatase A activity in human-metachromatic-leucodystrophy fibroblasts via retroviral-vector-mediated gene transfer.

Metachromatic leukodystrophy is a lysosomal storage disease caused by the deficiency of arylsulphatase A (ASA). A human ASA cDNA was subcloned into the retroviral vector pXT1. Replication-defective retrovirus was generated by transfection of the vector into the amphotropic packaging cell line PA317. Human fibroblasts from a patient suffering from metachromatic leucodystrophy was infected with the recombinant retrovirus. Infected fibroblasts expressed ten times more ASA compared with control fibroblasts from a normal individual. The ASA encoded by the integrated provirus was shown to be correctly transported into the lysosomes and to normalize the impaired degradation of cerebroside sulphate.

Cell Fractionation↗

PubMed2002

Isolated peripheral neuropathy in atypical metachromatic leukodystrophy: a recurrent mutation.

BACKGROUND: Metachromatic leukodystrophy (MLD) is a genetic neurodegenerative disorder resulting from a deficiency of arylsulfatase A. Late onset forms are relatively rare. Central nervous system (CNS) involvement is characteristic at all ages. METHODS: A patient in her late 40s with peripheral neuropathy was assessed by EEG, evoked potentials, CT and nerve conduction studies. Nerve and muscle biopsy samples were investigated by electron microscopy. Arylsulfatase A activity in leukocytes and excreted cerebroside sulfate were determined. The arylsulfatase A gene was investigated for mutations using polymerase chain reaction (PCR) and DNA sequencing. The identified mutation was expressed transiently in African green monkey kidney (COS) cells to determine the effect of the mutation on arylsulfatase A activity. RESULTS: Central nervous system functions were normal. Nerve conduction velocities were decreased. Sural nerve biopsy showed inclusions typical of MLD. Arylsulfatase A was less than 5% of normal. A homozygous mutation thr286pro was identified in the arylsulfatase A gene and demonstrated to be deleterious through transient expression studies. CONCLUSIONS: Our patient has a progressive peripheral neuropathy but has apparently intact CNS function at her present age of 57 years. Biochemical, physiological and pathological findings are consistent with a diagnosis of MLD. A homozygous mutation, thr286pro, found in her arylsulfatase A gene, decreased enzyme activity to a level consistent with a late onset form of MLD.

Animals↗