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  • 1
    ISSN: 1432-0533
    Keywords: Juvenile Amaurotic Idiocy ; Multilamellar, Crescentic, “fingerprint” ; Inclusions ; Lipofuscin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The electron microscopic findings of cerebral biopsies from two siblings with late infantile-juvenile amaurotic idiocy are presented. One patient had a mixture of “fingerprint” and multilamellar inclusions, often in the same cytosome. The second patient had only “fingerprint” inclusions. Electron microscopic findings in autopsied tissues from two other cases of late infantile-juvenile amaurotic idiocy with multilamellar cytosomes are also presented. Evolution of the multilamellar cytosomes into lipofuscin was not observed. We concluded that cases of late infantile-juvenile amaurotic idiocy with multilamellar and “fingerprint” inclusions should be distinguished from cases with lipofuscin.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 12 (1969), S. 103-115 
    ISSN: 1432-0533
    Keywords: Spongy Degeneration of the CNS ; Van Bogaert-Bertrand-Disease ; Electron Microscopy ; Astrocytic Mitochondria ; Abnormal Cerebral Edema
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über licht- und elektronenoptische Untersuchungen an Muskel-und Hirnbiopsien eines Falles von spongiöser Degeneration des ZNS berichtet. Die in der grauen und weißen Hirnsubstanz enthaltenen Vacuolen entsprechen a) Spalten in den Markscheiden infolge Aufsplitterung an der intraperiodischen Linie und b) geschwollenen Astrocytenperikaryen und-fortsätzen. Ungewöhnliche Mitochondrien mit Gehalt an kristallinem Material fanden sich nur in Astrocyten. Die ultrastrukturellen Befunde entsprechen denen des Hirnödems. Es wird angenommen, daß die Astroglia eine primäre Rolle in der Flüssigkeitsansammlung spielt, während die Markscheidenschwellung als eine Sekundärläsion aufgefaßt wird. Die mögliche Bedeutung abnormer Astrocyten-Mitochondrien wird diskutiert.
    Notes: Summary Light and electron microscopy study of skeletal muscle and cerebral biopsies from a case of spongy degeneration of central nervous system is reported. The multiple vacuoles present in cerebral gray and white matter correspond to (a) clefts within myelin sheaths resulting from splitting at the intraperiod line and (b) swollen astrocytic perikarya and processes. Unusual mitochondria containing crystalline-like material were observed only in astrocytes. The ultrastructural findings are consistent with cerebral edema. It is suggested that the astrocytes play a primary role in the fluid accumulation while the myelin swelling is a secondary lesion. The possible role of the abnormal astrocytic mitochondria is discussed.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 19 (1971), S. 265-270 
    ISSN: 1432-0533
    Keywords: Lipidosis ; Idiocy ; Cytoplasmic Inclusion ; Acid Phosphatase ; Residual Body
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Acid phosphatase reaction of the characteristic cytoplasmic organelle called “multilamellar cytosomes” (Gonataset al., 1968), in the biopsied cerebral cortex of a case of late infantile amaurotic idiocy, was studied with electron microscope. Occassionally positive enzyme reaction was found in these organelles. Smaller cytosomes or marginal and protruded parts of the larger ones showed positive acid phosphatase reaction, while the central parts of the larger cytosomes have little or no enzyme activity. This indicates the possibility that the fully developed multilamellar cytosomes may be a “residual body” (Novikoff, 1963; Novikoffet al., 1964; Novikoff, 1967), probably containing nonmetabolizable residues, and little or no acid phosphatase activity.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1211
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 122 (1965), S. 0 
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    [s.l.] : Nature Publishing Group
    Nature 214 (1967), S. 352-355 
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] Peculiar vesicular structures in the axons and synaptic terminals of neocortex of a 30 month old boy with psychomotor retardation of unknown origin are similar to the changes observed in other human diseases characterized by disorders of memory and learning. Such changes in the architecture of ...
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 44 (1985), S. 0 
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: The epitopes (antigenic sites) for seven monoclonal antibodies (MAbs) evoked in rats or mice by guinea pig or monkey myelin basic protein (BP) have been located in four different sequences of the BPs extracted from various species. Six of the MAbs were evoked by guinea pig BP. (1) One epitope, possibly a pair, is included within residues 1–14 of all BPs tested and reacts with two rat IgG MAbs. (2) A definite pair of over-lapping epitopes includes the central Phe91-Phe92 sequence. One epitope is contained entirely within sequence 90–99 and reacts with a rat IgG MAb. The substitution of Ser in chicken BP for Thr97 destroys this epitope. The other epitope appears to include residues on the amino side of Phe44 and even of His32 and suggests some tertiary structure in BP. This epitope reacts with a mouse IgM MAb that does not recognize the chicken substitution. (3) The third epitope lies within residues 114–121, specifically including Trp118, and reacts with a rat IgG MAb. A cross-reacting epitope probably includes residues 44–45 in certain species (guinea pig and bovine but not rabbit). (4) Another pair of epitopes is located within residues 131–140 but is severely species-restricted. This region in guinea pig BP evoked a species-specific mouse IgM MAb. The same region in monkey BP evoked the seventh MAb, a mouse IgG, which reacts with human, chimpanzee, monkey, bovine, and rat-18.5 kDa BPs and to a lesser extent rabbit BP but not with guinea pig, pig, or chicken BPs. Some tertiary structure in guinea pig BP is also suggested by the reactivities with the IgM MAb. All of the MAbs react with myelin in histologic preparations, but the optimum method of preparation of the tissue varies with each.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 45 (1985), S. 0 
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: We report a method for the isolation of enriched fractions of intact Golgi apparatus from neurons of 10- to 12-day-old rat brains. Neurons were prepared according to a modified method of Farooq and Norton [J. Neurochem.31, 887–894 (1978)]. Golgi-enriched fractions were obtained after centrifugation of postmitochondrial supernatants in a discontinuous sucrose gradient. Golgi fractions 1 and 2, recovered at the interfaces of 28–34% and 34–36% sucrose densities, respectively, were examined with morphometric and enzymatic methods. Morphometric analyses showed that 21–34% of fraction 1 and 11–29% of fraction 2 consisted of intact Golgi apparatus. Lysosomes, mitochondria, ribosomes, and rough endoplasmic reticulum contaminated fraction 1 (6–10%) and fraction 2 (14–26%). Golgi fraction 1 showed a 25- to 65-fold enrichment over neurons of UDP Gal:GlcNAc galactosyltransferase, CMP-sialic acid:lactosylceramide sialyltransferase, and PAPS:cerebroside sulfotransferase activities. Golgi fraction 2 showed a 8-to 23-fold enrichment over neurons of the activities of the above glycolipid- and glycoprotein-synthesizing enzymes. The activities of the possible marker enzymes rotenone-insensitive NADH-cytochrome c reductase, succinate-cytochrome c reductase, and arylsulfatase were low or minimally elevated in the Golgi fractions. A sevenfold enrichment of Na+,K+-ATPase activities was found in the Golgi fractions. This is consistent either with significant plasma membrane contamination or with the presence of this enzyme in the neuronal Golgi apparatus.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    [s.l.] : Nature Publishing Group
    Nature 208 (1965), S. 1338-1339 
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] To our knowledge, ultrastructural investigations in. this disease have not been made. This electron microscopic examination of a cortical biopsy from a case of subacute sclerosing encephalitis has revealed a large number of viruslike particles in the cytoplasm of astrocytes in the white matter; ...
    Type of Medium: Electronic Resource
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