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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 331 (1958), S. 287-313 
    ISSN: 1432-2307
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Zusammenfassung 1. Den zehn im Weltschrifttum bekannten autoptisch gesicherten Fällen von neuromuskulärer (Selberg) sive myatoner (Hamperl) Form der Glykogenspeicherungskrankheit wird eine eigene Beobachtung hinzugefügt. 2. Bei dem 5 1/2 Monate alten dystrophen weiblichen Säugling lag eine schwere generalisierte Glykogenose mit Beteiligung des Nerven systems, der quergestreiften Muskulatur, des Herzens (120 g), der glatten Muskulatur (Gefäße, Bronchial- und Darmmuskulatur), der Leber (275 g), der Nieren, der Haut, der Nebennierenrinde, des Pankreas und gering des RHS vor. 3. In Übereinstimmung mitSelberg- wird der Begriff der Ence-phalomyelopathia glycogenica geprägt. Sie ist durch eine generalisierte, aber regional und cellular außerordentlich wechselnde intraprotoplasmatische Glykogenspeicherung der Nerven- und Gliazellen, eine gestörte Markscheidenreifung, eine diffuse Sklerose (isomorphe Fasergliose) und durch Merkmale einer leicht retardierten Entwicklung gekennzeichnet. 4. Für die Glykogenspeicherung des Nervensystems läßt sich einePathoklisenreihe aufstellen. An der Spitze stehen motorische Vorderhorn- und Hirnnervenzellen, große Spinalganglienzellen, Nucleus basalis Meynert. Mittelgradigen Glykogengehalt besitzen in absteigender Folge unter anderem: Pallidum, Makroglia, Myelinisationsglia, Zellen des peripheren vegetativen Nervensystems, Hypothalamus, Thalamus, Striatum, Pyramidenzellen, Mikroglia. Von der Glykogenspeicherung bleiben praktisch ausgespart: Körnerzellen der Klein- und Großhirnrinde, Dies Gyrus dentatus, kleine Striatumzellen und Purkinje-Zellen. Verteilungsschema weicht von dem der Lipoidosen deutlich ab. 5. Es wird die Hypothese vertreten, daß eine lokale Schädigung, wahrscheinlich im Sinne einer cellulären Hyp- oder Dysenzymatose, den entscheidenden pathogenetischen Faktor für die Glykogenspeicherung im Nervensystem darstellt.
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  • 2
    ISSN: 1432-2307
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary In the neuromuscular (myatonic) type of glycogenosis the degenerated muscle-fibers contain in vacuoles a granular „basophilic substance“ (BS), behaving in many pointslike a free acid mucopolysaccharide (Dark). The water- and acid-soluble substrate is free from lipids and very probably free from protein; extraction with diastase and hyaluronidase is without effect. With the periodic-acidSchiff, Astra-blue,Hale and aldehyde-fuchsin method (without preliminary oxidation) the BS is well demonstrable. It shows a strong, relatively alcohol-fast metachromasia. The dye-binding capacity for methylene blue (MBE,Pearse) disappears at pH 2,4. The Astra-blue and aldehyde-fuchsin affinity and the failing methylation, are discussed. - The precise chemical constitution and the manner of origin of the BS remain unknown.
    Notes: Zusammenfassung Bei der neuromuskulären (myatonen) Form der Glykogenosen zeigen die schwer degenerierten quergestreiften Muskelfasern eine spezifische intravacuolär eingelagerte, körnig-krümelige „basophile Substanz“ (BS), die sich in Übereinstimmung mitDark in vielen Punktenwie ein freies saures Mucopolysaccharid verhält. Das wasser- und besonders säureempfindliche Substrat ist lipid- und sehr wahrscheinlich auch proteinfrei. Diastase- und Hyaluronidase-Extraktion bleiben erfolglos. Mit der PJS-, Astrablau-, Hale- und Aldehydfuchsin-Methode (ohne Voroxydation) ist die BS gut darstellbar. Sie weist eine kräftige, relativ alkoholbeständige Metachromasie auf. Die Methylenblaubindefähigkeit (MBE,Pearse) erlischt erst unter pH 2,4. Die Astrablau- und Aldehydfuchsinaffinität sowie die bemerkenswerterweise nicht zu erzielende Methylierung sind unter anderem Gegenstand einer ausführlichen Diskussion.-Über die genaue chemische Konstitution und den Entstehungsmodus können vorerst keine Aussagen gemacht werden.
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  • 3
    ISSN: 1432-2307
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The organs of a 4 1/2 year old boy with the typical signs of gargoylism were studied histochemically and biochemically. Besides gangliosides, phospholipids, acid mucopolysaccharides (in part labile to testicular hyaluronidase) and glycoproteins,strongly PAS positiveBialnegative acidglycolipids were also determined. The ganglioside content of the cerebral cortex was only 0.65% (dry weight). Cholesterol was demonstrated for the first time, chiefly in the free form, in the lipid complex of nerve cells and foam cells of the spleen and renal tubuli. The composition of the stored substances was dependent upon fluctuations associated with organs and certain cells. The histologie changes of the cerebrospinal and autonomic ganglionic cells, the fasciculi optici, and the plexus chorioidei were unusual. The disease is regarded as a consequence of a gene regulated generalized disturbance of the sphingoglycolipid and polysaccharide metabolism.
    Notes: Zusammenfassung Die Organe eines 4 1/2 Jahre alten Knaben mit den typischen Merkmalen des Gargoylismus wurden histochemisch und biochemisch untersucht. Neben Gangliosiden, Phospholipiden, sauren (teils gegen Testis-Hyaluronidase labilen) Mucopolysacchariden und Glykoproteiden waren auchstark PJS-positive, Bialnegative saureGlykolipide beteiligt. Der Gangliosidgehalt der Hirnrinde betrug nur 0,65% (bezogen auf Trockengewicht). Erstmalig wurde Cholesterin, vorwiegend in freier Form, im Lipidkomplex der Nervenzellen, Schaumzellen der Milz und Nierentubuli nachgewiesen. Die Zusammensetzung der Speicherungsgemische unterlag organ- und zellspezifischen Schwankungen. Die histologischen Veränderungen der cerebro-spinalen und vegetativen Ganglienzellen, der Fasciculi optici und der Plexus chorioidei waren ungewöhnlich. Die Erkrankung wird als Folge einer genbedingten generalisierten Störung des Sphingoglykolipid- und Polysaccharid-Stoffwechsels angesehen.
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  • 4
    ISSN: 1432-0533
    Keywords: Keywords Alzheimer’s disease ; Down’s syndrome ; β-Amyloid precursor protein ; β-Amyloid peptide
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Strong genetic evidence has been accumulated in favor of a central role of β-amyloid precursor protein (APP) and β-amyloid peptide (βA4) in the pathogenesis of Alzheimer’s disease (AD). We employed four newly developed APP and βA4 antibodies and performed a comparative neuropathological study of patients with Down’s syndrome (DS), early-onset familial AD and sporadic AD to investigate the distribution of APP and βA4 plaque densities in the cerebral cortex of these disorders. Quantitative analysis of APP versus βA4 plaques revealed that brains with early-onset familial AD and sporadic AD showed significantly more βA4 plaques than brains with DS (P 〈 0.05). In contrast, APP plaques were more abundant in DS cerebral cortex (P 〈 0.02). These observations suggest that the development of pathological changes in DS brains does not parallel that observed in AD, which might be attributable to different causes in the pathogenesis of βA4 formation. A comparison of these disorders may be useful to further complement our knowledge of the mechanisms leading to plaque development.
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  • 5
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] The removal of apoptotic cells is essential for the physiological well being of the organism. In Caenorhabditis elegans, two conserved, partially redundant genetic pathways regulate this process. In the first pathway, the proteins CED-2, CED-5 and CED-12 (mammalian homologues CrkII, Dock180 and ...
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  • 6
    ISSN: 1617-4623
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Summary The partially circularly permuted, terminally redundant structure of the DNA of phage ϕH has been confirmed by a cleavage map for the restriction enzymes PstI, ClaI, BglII, HindIII, and, partially, BamHI. Six variants of phage ϕH have been isolated from 71 single plaques. Their genomes differ by several insertions, a deletion, and an inversion of a DNA segment with a minimal length of 11 kb. The inversion occurs with high frequency in variants carrying at the flanks of the invertible DNA in verted repeats of a 1.8 kb DNA element which shares sequence homology with the DNA of H. halobium and may be involved in the extreme variability of its genome.
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  • 7
    Electronic Resource
    Electronic Resource
    [s.l.] : Macmillan Magazines Ltd.
    Nature 390 (1997), S. 294-298 
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] In Caenorhabditis elegans, the early embryo contains five somatic founder cells (known as AB, MS, E, C and D) which give rise to very different lineages. Two simply produce twenty intestinal (E) or muscle (D) cells each, whereas the remainder produce a total of 518 cells which collectively ...
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  • 8
    Electronic Resource
    Electronic Resource
    [s.l.] : Macmillian Magazines Ltd.
    Nature 412 (2001), S. 202-206 
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] Genetic studies have identified over a dozen genes that function in programmed cell death (apoptosis) in the nematode Caenorhabditis elegans. Although the ultimate effects on cell survival or engulfment of mutations in each cell death gene have been extensively described, much less is ...
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  • 9
    Electronic Resource
    Electronic Resource
    [s.l.] : Nature Publishing Group
    Nature 385 (1997), S. 588-589 
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] Cowing and Kenyon1 raise an important issue concerning Hox genes by suggesting that these genes may not only be expressed according to position2 but also according to cell lineage. This suggestion is based on two experiments in which the so-called M cell and two hypodermal V6 cells were manipulated ...
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  • 10
    Electronic Resource
    Electronic Resource
    New York, NY : Wiley-Blackwell
    BioEssays 18 (1996), S. 591-594 
    ISSN: 0265-9247
    Keywords: Life and Medical Sciences ; Cell & Developmental Biology
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Biology , Medicine
    Notes: Recent findings suggest that C. elegans, albeit displaying an invariant cell lineage for embryonic development, uses the same basic strategy for embryogenesis as other organisms. The early embryo is regionalised by cell-cell interactions.
    Additional Material: 2 Ill.
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