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  • Neuromuskuläre Erkrankungen  (2)
  • ADHESION, ALLOGRAFTS, ALPHA(V)BETA(3), ALPHA(V)BETA(3) INTEGRIN, ALPHA-V-INTEGRINS, ANTAGONIST, ARRE  (1)
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  • 1
    Keywords: ADHESION, ALLOGRAFTS, ALPHA(V)BETA(3), ALPHA(V)BETA(3) INTEGRIN, ALPHA-V-INTEGRINS, ANTAGONIST, ARRE
    Abstract: Integrin-mediated cell adhesion and signaling is essential to vascular development and inflammatory processes. Elevated expression of integrin alpha(v)beta(3) has been detected in ischemia-reperfusion injury and rejecting heart allografts. We thus hypothesized that the inhibition of alpha(v)-associated integrins may have potent anti-inflammatory effects in acute kidney allograft rejection. We studied the effects of a peptidomimetic antagonist of alpha(v) integrins in two rat models of renal allotransplantation, differing in degree of major histocompatibility complex mismatch. Integrin alpha(v)beta(3) was up-regulated in rejecting renal allografts. Integrin antagonist reduced the histological signs of acute rejection, the intensity of the mononuclear cell infiltration, and cell proliferation in the grafted kidneys. This could be correlated to a reduced leukocyte-endothelial interaction and an improved peritubular microcirculation observed by intravital microscopy. In vitro under laminar flow conditions, the arrest of monocytes to interleukin-1 beta-activated endothelium was decreased. Furthermore, in co-culture models the proliferation and transmigration of monocytes/macrophages, endothelium, and fibroblasts induced by renal tubular epithelia was efficiently inhibited by alpha(v) integrin antagonism. These data reveal an important role of this integrin subclass in leukocyte recruitment and development and maintenance of acute rejection; blockade of alpha(v) integrins may provide a new therapeutic strategy to attenuate acute allograft rejection
    Type of Publication: Journal article published
    PubMed ID: 17702892
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  • 2
    ISSN: 1432-1440
    Keywords: Neuromuscular disorders ; Congenital myopathy ; Central core disease ; “structured cores” in type II-fibers ; Neuromuskuläre Erkrankungen ; Congenitale Myopathie ; Central core disease ; „structured cores“ in Type II-Fasern
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über einen sporadischen Fall einer congenitalen langsam progredienten neuromuskulären Erkrankung berichtet. Die im Rahmen der Muskelbiopsie zu erhebenden lichtmikroskopischen, ultrastrukturellen und enzymhistochemischen Befunde entsprechen einer Central Core Disease mit „structured cores” in Typ II-Fasern.
    Notes: Summary Report on a sporadic case with congenital slowly progressive neuromuscular disease. Light microscopic, ultrastructural and histochemical changes of muscle biopsy reveal the characteristics of central core disease with “structured cores” in type II-fibers.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1440
    Keywords: Neuromuscular disorders ; Centronuclear myopathy ; Type I-fiber atrophy ; “Myotubelike structures” ; Neuromuskuläre Erkrankungen ; centronucleäre Myopathie ; Typ I-Faser Atrophie ; “Myotube-like structures”
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über eine Familie berichtet, in welcher sowohl die Mutter, als auch ihre beiden Töchter an einer congenitalen, langsam progredienten neuromuskulären Erkrankung leiden. Die im Rahmen der Muskelbiopsie zu erhebenden histologischen, histochemischen und ultrastrukturellen Befunde zeigen bei der Mutter das Vollbild der sog. centronucleären Myopathie. Die charakteristischen zentralständigen Kerne mit den umgebenden pericentronucleären Strukturanomalien der Fasern werden nahezu in allen Muskelfasern angetroffen. Bei den Töchtern dagegen sind morphologisch fast ausschließlich die Typ I-Fasern befallen, welche zusätzlich im Durchmesser kleiner als normal erscheinen. Der Nachweis dieser beiden Formen von centronucleärer Myopathie in einer Familie beweist, daß hier offenbar zwei morphologische Varianten, wahrscheinlich sogar Stadien eines Krankheitsbildes vorliegen. Das zusätzliche Vorhandensein einiger weiterer struktureller Besonderheiten (rod bodies, core-ähnliche Areale) unterstreicht die Verwandtschaft der Erkrankung mit anderen congenitalen langsam progredienten Myopathien (nemaline myopathy, central core disease).
    Notes: Summary This report deals with a family in which the mother and her two daughters suffer from a congenital, slowly progressive neuromuscular disease. Histological, histochemical and ultrastructural observations of the mother's muscle biopsy reveal the characteristics of the centronuclear myopathy. In this case central nuclei and pericentronuclear abnormalities of muscle fibers are found in almost all fibers. Biopsies obtained of the two daughters show alterations especially of type I-fibers, which often are smaller than normal. The presence of these two forms of centronuclear myopathy in one family indicates that these may be only different morphological types or states of one illness. Additionally, other structural findings (rod bodies, core like regions) emphasize the similarities with other congenital slowly progressive myopathies (nemaline myopathy, central core disease).
    Type of Medium: Electronic Resource
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