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  • 1
    Electronic Resource
    Electronic Resource
    COMPOSITION OF CEREBRAL LIPIDS IN MURINE LEUCODYSTROPHY: THE QUAKING MUTANT (1970)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 17 (1970), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The composition of sphingolipids and phospholipids of mouse brain during myelination was determined in the Quaking mutant, which manifests a genetic disorder of myelin formation, and in littermate controls. The biochemical changes during myelination in the brains of the controls corresponded quantitatively with previous findings in a different strain of mice. The Quaking mutant exhibited concentrations of sphingolipids and phospholipids in brain which were comparable to those of controls in the early stage of myelination but the tissue content failed to increase with maturation. The greatest differences occurred in the cerebrosides which at 65 days of postnatal age were only 10 per cent of control levels. During development the pattern of cerebral levels of sphingomyelin, plasmalogen and total phospholipid in the mutants tended to resemble that of the cerebrosides.The defect in the Quaking mutant is compatible with a failure in maturation of myelin. These findings have been compared with those in the Jimpy mutant, a different genetic disorder of myelin in the mouse previously studied in a similar fashion. The Jimpy mutant is characterized by a quantitatively more pronounced deficiency of myelin lipids and a decline in cerebrosides during brain development.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1970.tb03370.x
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  • 2
    Electronic Resource
    Electronic Resource
    COMPOSITION OF CEREBRAL LIPIDS IN MURINE SUDANOPHILIC LEUCODYSTROPHY: THE JIMPY MUTANT (1970)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 17 (1970), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract— The composition of sphingolipids and phospholipids of mouse brain during myelination was determined in normal animals and in mice with a genetically-determined disorder of myelin formation. Myelination was normally characterized by a two-fold increase in total phospholipids of brain, a four-fold increase in total sphingolipids, and a six-fold increase in cerebrosides. The Jimpy mutant, with defective formation of myelin in the central nervous system, demonstrated a marked deficiency of cerebrosides and a significantly lower content of total sphingolipids, without alteration of the composition of phospholipids. The increasing content of cerebrosides in the brains of the leucodystrophic mutant at the time in development when myelination is most active and the subsequent relative deficit suggest that the failure of myelin formation is not the result of a defect in biosynthesis of cerebrosides.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1970.tb00503.x
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    FATTY ACID COMPOSITION OF CEREBROSIDES, SULPHATIDES AND CERAMIDES IN MURINE LEUCODYSTROPHY: THE QUAKING MUTANT (1972)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 19 (1972), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract— The fatty acid composition of cerebrosides, sulphatides and ceramides has been determined at 20 days postpartum in the brains of Quaking mutant mice and of littermate controls. There was a significant deficit in the proportion of long-chain fatty acids (C22-C24) affecting both normal and a-hydroxy fatty acids of the cerebrosides. The proportion of normal but not the a-hydroxy long-chain fatty acids of the sulphatides was also decreased. Striking and disproportionate deficits of the C24:1 and C24 h:1 fatty acids of cerebrosides, sulphatides and ceramides characterized the brain of the Quaking mutant, and an increased proportion of C23 h:O fatty acid was found in the cerebrosides and sulphatides of the brain of this mutant. We compared these data with findings on the Jimpy mutant which has been examined by the same techniques. The deficiency of long-chain fatty acids which was found in the cerebrosides and sulphatides of both mutants was less extensive but more selective in the Quaking mutant.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1972.tb01340.x
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    FATTY ACID COMPOSITION OF CEREBROSIDES, SULPHATIDES AND CERAMIDES IN MURINE SUDANOPHILIC LEUCODYSTROPHY: THE JIMPY MUTANT (1971)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 18 (1971), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The fatty acid composition of cerebrosides, sulphatides and ceramides was determined at 15-16 days post partum in the brain of the Jimpy mutant and in littermate controls. There was a marked deficit in the long chain fatty acids (C22-C24) of cerebrosides and sulphatides of Jimpy brain, with the unsubstituted fatty acids affected more than the alpha-hydroxy fatty acids. A decrease of long chain normal fatty acids was also found in the ceramides of Jimpy brain. The deficit of long chain fatty acids in these sphingolipids of the Jimpy brain was more severe than that found in the Quaking mutant which has a less extensive disorder of myelin formation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1971.tb03737.x
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  • 5
    Electronic Resource
    Electronic Resource
    Schilder's diffuse sclerosis: A biochemical and ultrastructural study of myelinoclastic demyelination (1972)
    Springer
    Acta neuropathologica 20 (1972), S. 85-95 
    Details
    ISSN: 1432-0533
    Keywords: Lipid Composition ; Axonal Loss ; Ganglioside Deficit ; Schilder's Disease ; Electron Microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of Schilder's diffuse sclerosis with a four-year history of progressive neurologic deterioration in a boy dying at ten years of age is reported. The asymmetry of the process was indicated during life by the clinical findings. A unicentric focus of demyelination was found with the initial stages in the occipital and temporal white matter and a subacute reaction in the rostral cerebral white matter, brain stem, and cerebellum. Electron microscopic study confirmed the extensive axonal loss. No particles compatible with a virus structure were identified. Analysis of the regional composition of cerebral lipids demonstrated large deficits of cerebroside, sulfatide, cholesterol, total phospholipid, sphingomyelin, and ganglioside in the occipital lesion. A significant but less complete deficit of lipids was found at the lesion margin in frontal white matter. An increase of cholesterol esters was demonstrated at this site as well. The normal-appearing white matter of rostral frontal lobe showed a decreased level of ganglioside and normal concentrations of the other structural lipids. This decreased ganglioside concentration of grossly normal white matter suggest the advisability of considering Wallerian degeneration in interpreting the analytical data obtained in the study of the demyelinating disorders. The morphological and biochemical findings indicate the progressive spread of the axonolytic and myelinolytic process in the pathogenesis of Schilder's diffuse sclerosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00691126
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    Paper (German National Licenses)
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