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  • 1
    Electronic Resource
    Electronic Resource
    Niemann-Pick disease (Crocker's type C) (1975)
    Springer
    Acta neuropathologica 33 (1975), S. 191-200 
    Details
    ISSN: 1432-0533
    Keywords: Niemann-Pick disease ; Storage distribution ; Cerebral vascular glycolipid ; Histochemistry ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A histochemical study is reported of regional differences of the lipid storage in a case of Niemann-Pick disease (NPD) type C. Besides tissues known to be affected (reticuloendothelium, hepatocytes, nervous system), storage was demonstrated in adrenal cortical spongiocytes, sweat glands, renal glomerular and tubular cells, smooth muscle, excretory tubules of some salivary glands, ependyma and in choroid plexus. In most tissues were stored sphingomyelin, cholesterol and a small amount of a glycosphingolipid. In the endothelium of cerebral and spinal vessels the main stored lipid was a glycosphingolipid. The significance of these regional differences are discussed and their study is recommended as a useful counterpart to the biochemical investigation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688393
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Niemann-Pick disease (1980)
    Springer
    Virchows Archiv 385 (1980), S. 215-231 
    Details
    ISSN: 1432-2307
    Keywords: Sphingomyelinase deficit ; Niemann-Pick disease ; Liver ; Histochemistry ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The results of a complex analysis of liver tissue are presented (four biopsy and two autopsy samples) obtained from six patients with Niemann-Pick disease (NPD) with a gross deficiency of sphingomyelinase (SMase) accompanied by a typical increase in sphingomyelin (SM). There were five cases of NPD type A (four of them with an atypical, prolonged course) and one case of type B. By means of lipid histochemistry it was possible to demonstrate SM storage both in hepatocytes and in the reticuloendothelial system (RES) of the liver (Kupffer cells and portal macrophages) and to show in two siblings with NPD type A a so-far undescribed centrilobular storage pattern. Enzyme histochemistry revealed a secondary deficit of nonspecific esterase activity and acid β-galactosidase in liver storage macrophages and varying degrees of suppression of hepatocytic enzyme activities as a reaction to lipid storage of sudden onset. Ultrastructurally, it was possible to demonstrate cholesterol in lysosomes by using digitonin fixation, the involvement of Ito cells in lipid storage, the aggregation of storage lysosomes with certain other organelles and their occasional connections with the endoplasmic reticulum. The problems of possible lipid extraction during processing were considered as a cause of pronounced lysosomal electron-lucidity and of the ultrastructural identification of the participating lipopigment. The significance of the findings is discussed in relation to the existing classification and, particularly, to the stored lipid dilemma of cases of NPD type C.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00427406
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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