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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 64 (1986), S. 197-205 
    ISSN: 1432-1440
    Keywords: Alpha-1 antitrypsin ; Heterozygous deficiency ; Chronic liver disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Homozygous deficiency of alpha-1 antitrypsin is the most common inborn error or metabolism in Europe. Severe deficiency of this major protease inhibitor in serum is associated with chronic obstructive lung disease, chronic liver disease in adults and neonatal hepatitis. An overview is given of the role of heredity, and the diagnostic criteria and clinical and histological findings in this disorder. Emphysema seems to be caused by the free elastolytic activity of white cells, leading to the degradation of elastin. The pathophysiology of liver disease — less well understood — is discussed with special emphasis on the importance of heterozygous alpha-1 antitrypsin deficiency. Exogenous noxae seem to play an important role in the pathogenesis of heterozygous deficiency. In view of the 7% frequency of heterozygous alpha-1 antitrypsin deficiency in the European population and the role of noxae in the development of pulmonary and liver diseases, improved prophylaxis is mandatory.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Research in experimental medicine 170 (1977), S. 271-281 
    ISSN: 1433-8580
    Keywords: Arterial wall ; Blood vessels ; Lysosomes ; Lysosomal Enzymes ; Matrix vesicles ; Arterienwand ; Blutgefäße ; Lysosomen ; Lysosomale Enzyme ; Matrix-Vesikel
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Der elektronenmikroskopisch geführte Nachweis saurer Phosphatase in Matrix-Vesikeln (=extrazellulären membranbegrenzten Formationen mit heteromorphem Inhalt), die vermehrt in der Wand hämodynamisch fehlbelasteter Arterien auftreten, regte biochemische Untersuchungen zur Prüfung der Aktivitäten lysosomaler Enzyme in der Wand stillgelegter Arterien an. Ausgewertet wurden Hodenarterien der Ratte unter den Bedingungen der Nullfunktion 4, 5, 7, 13 und 14 Tage nach Ligatur. Innerhalb dieses Zeitraums kommt es in den untersuchten Arterienstücken zu einer Aktivitätszunahme der lysosomalen Glykosidasenβ-N-acetyl-glucosaminidase undβ-Galaktosidase um 40–70% im Vergleich zur unbehandelten A. testicularis der Gegenseite. Die grundlegende Bedeutung der extrazellulären Lysosomen für normale und pathologische Umbauvorgänge in der Gefäßwand wird erörtert.
    Notes: Summary The electron microscope demonstration of reaction products of the acid phosphatase within matrix vesicles (=extracellular membrane-bound corpuscles) of the wall of hemodynamic or metabolic imbalanced arteries stimulated experiments about the activities of lysosomal enzymes in the wall of wrongly loaded arteries. In our case arteries without any hemodynamic function (=“null function”) were investigated 4, 5, 7, 13 and 14 days after applying a ligature. In relation to the nonligated testicular arteries of the other side the wall of the ligated vessels show an increase of lysosomal glycosidases (β-N-Acetyl-glucosaminidase andβ-galactosidase) about 40–70%. The significance of the extracellular lysosomes for transformations of the vessel wall under physiological and pathological conditions is discussed.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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