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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 40 (1977), S. 95-98 
    ISSN: 1432-0533
    Keywords: Microcephaly ; Pelizaeus-Merzbacher disease ; Patchy demyelination ; Anoxic leukoencephalopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The neuropathological studies of a 4-year old idiot with microcephaly revealed a striking demyelinating disease compatible with Pelizaeus-Merzbacher disease. The case seemed to be best classified as the patchy demyelination type, but the most unusual finding was a prominent vasculature in some demyelinated areas. Intrauterine hypoxia was proposed as a causative factor of this type of demyelinative lesions
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 26 (1976), S. 0 
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: —Recent clinical and morphological evidence established that adrenoleukodystrophy is a distinct X-linked genetic disorder. Fatty acid compositions of lipids in the brain, adrenal and serum from seven patients were examined. Cholesterol esters of both brain and adrenal contained substantial proportions of fatty acids longer than C22 (11.8–41.9% of total in the brain and 13.4-34.8% of total in the adrenal), while cholesterol esters from normal and pathological control specimens contained very little. These very long chain fatty acids were generally saturated in brain cholesterol esters but significant amounts of unsaturated long chain fatty acids were also present in adrenal cholesterol esters. The long chain fatty acids showed bell-shaped distribution with C25 or C26 at the peak. Ganglio-sides from patients’white matter also showed increased proportions of very long-chain fatty acids, up to 50% of the total. Qualitatively similar but much milder fatty acid abnormalities were also found in galactosylceramide of the brain. On the other hand, fatty acids and fatty aldehydes of brain glycerophospholipids, adrenal free fatty acids, triglycerides and glycerophospholipids were not abnormal. Furthermore, serum cholesterol esters from two patients did not show the long-chain fatty acid abnormality found in brain and adrenal cholesterol esters. Sequential extractions with acetone and hexane established that the characteristic birefringent material in the brain and adrenal is indeed cholesterol esters with very long chain fatty acids. This type of fatty acid abnormality has not been described in other pathological conditions and may well represent the unique biochemical abnormality that is directly related to the fundamental genetic defect underlying adrenoleukodystrophy.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 27 (1976), S. 0 
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 27 (1976), S. 0 
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract— Activity of cholesterol ester hydrolase localized almost exclusively in the myelin sheath (Eto & Suzuki, 1973a) was greatly affected by exogenous lipids added to the assay mixture. With isolated myelin as the enzyme source, phosphatidylserine was most effective in stimulating the activity. Other phospholipids were less effective. Efhanolamine phospholipid was slightly inhibitory and lysolecithin was strongly inhibitory. Differences in the fatty acid composition did not appear to account for such different effects. Glucosylceramide, galactosylceramide and digalactosylceramide were stimulatory while sulfatide, ganglioside and its asialo-derivative were inhibitory. Saturated fatty acids were generally stimulatory while corresponding unsaturated acids were strongly inhibitory. In order for exogenous lipids to be effective they had to be added to the assay mixture as free dispersion. When heat-inactivated myelin was used as the lipid source, no effect was observed, while equivalent amounts of a whole white matter lipid mixture was effective. Although phosphatidylserine was the most effective activator among the lipids tested, it could not completely replace sodium taurocholate present in the standard assay system. When isolated myelin was stored frozen, the activity of the enzyme declined gradually in the standard system without additional lipids. The stimulating effect of phosphatidylserine was greater for such partially inactivated enzyme sources, although it did not completely restore the activity to that of fresh preparations. When myelin was fractionated into basic protein, proteolipid protein and the high molecular weight acidic protein (Wolfgram) fractions, the last fraction contained most of the recovered activity. However, Wolfgram protein was less active than the intact myelin when assayed without additional lipid. The addition of phosphatidylserine completely restored the activity of this partially delipidated preparation.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 28 (1977), S. 0 
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract— The cholesterol ester hydrolase of rat brain, localized almost exclusively in the myelin sheath, has been solubilized from the acidic high-molecular-weight protein fraction of purified myelin. Solubilization required both high ionic strength and an amphoteric detergent, Miranol H2M. Solubilized preparations with apparent purification factors of 300–500 fold over the starting homogenate still contained approx 25% lipid but were retarded on the Sephadex G-200 column. The enzyme was reversibly precipitated when the concentration of either Miranol H2M or KCI was lowered below certain critical levels. The soluble enzyme was characterized for the pH optimum, linearity against incubation time and enzyme protein, and apparent Km. Activity was dependent on the presence of exogenously added lipid. Phosphatidylserine at optimum concentrations stimulated the hydrolytic activity 25-Fold. Effects of other lipids, bile salts, cations, heating and potential inhibitors were examined. β-Naphthyl oleate was a competitive inhibitor but both β-naphthyl acetate and cholesteryl butyrate were non-competitive inhibitors. These results suggested a heterogenous nature of the rat myelin cholesterol ester hydrolase, possibly with different specificities with respect to the chain length of the acyl group of substrates.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Infection 7 (1979), S. S255 
    ISSN: 1439-0973
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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