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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 59 (1981), S. 83-90 
    ISSN: 1432-1440
    Keywords: Collagen ; Marfan's syndrome ; Fibroblasts ; Aortic-aneurysm ; Kollagen ; Marfan-Syndrom ; Fibroblasten ; Aorten-Aneurysma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Der Fallbericht einer 33jährigen Frau mit einem ausgeprägtem Marfan-Syndrom wird vorgestellt. Sie hatte typische ossäre, okuläre, kardiovaskuläre und auch pulmonale Veränderungen, auf deren Bedeutung näher eingegangen wird. Wegen einer schweren Aorteninsuffizienz bei einem Aneurysma der Aorta ascendens und einer beträchtlichen Mitralinsuffizienz wurde ein Doppelklappenersatz durchgeführt und eine Prothese der Aorta ascendens eingesetzt. Die Prognose, die diagnostischen und therapeutischen Möglichkeiten der lebensbedrohlichen kardiovaskulären Komplikationen bei Marfan-Syndrom werden diskutiert. Das bei der Operation gewonnene Aortengewebe wurde histologisch und biochemisch analysiert. Histologisch zeigte sich eine typische Medianekrose. Biochemisch (Fibroblastenkultur, Elektronenmikroskopie) fand sich eine veränderte Zusammensetzung der kollagenen Anteile. Sowohl in der Adventitia wie in der Media war Kollagen Typ I nahezu völlig zugunsten von Kollagen Typ III verschwunden. Diese Verschiebung galt in gleicher Weise für die Prokollagene. In der Haut der Patientin fand sich jedoch eine normale Verteilung von Prokollagen und Kollagen der Typen I und III. Die weitgehende Reduktion von Kollagen Typ I stellt möglicherweise die Ursache der Wandschwächung der Aorta dar, die die Ursache der Aneurysmabildung und der Aorteninsuffizienz ist.
    Notes: Summary The case report on a 33 year old woman with prominent features of Marfan's syndrome is presented. Characteristic signs were seen in the bones, the eyes, the cardiovascular system, and the lungs. Due to regurgitation of both the aortic and mitral valves and an aneurysm of the ascending aorta a double valve replacement was made, including a prothesis of the aorta. The problems of early diagnosis and therapy of the life-threatening cardiovascular complications are discussed. Tissue specimens from the aorta were analysed histochemically and biochemically. Histology showed a typical necrosis of the media with cyst formation. Biochemical analysis by in vitro labeling of collagen in tissue explants and by electon microscopical evaluation showed proportions of type I and type III collagen which were significantly different from controls. In both the media and the adventitia the amount of type I collagen was drastically reduced as shown by quantitation of collagen and procollagen. Fibroblasts derived from the skin of the patient showed a normal content of type I and type III collagen. It is conceivable that the reduced content of type I collagen in the aortic wall is responsible for the weakness of the vessel wall causing formation of aneurysm and its sequelae.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1420-9071
    Keywords: Bradyrhizobium ; communication ; competitiveness ; Rhizobium ; vesicular-arbuscular (VA) mycorrhiza fungi
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract After a short summary on the ecology and rhizosphere biology of symbiotic bacteria and vesicular-arbuscular (VA) mycorrhiza fungi and their application as microbial inocula, results on competitiveness and communication are summarized. Stress factors such as high temperature, low soil pH, aluminium concentrations and phytoalexins produced by the host plants were studied withRhizobium leguminosarum bv.phaseoli andRhizobium tropici onPhaseolus beans. Quantitative data for competitiveness were obtained by usinggus + (glucoronidase) labelled strains, which produce blue-coloured nodules. ForPhaseolus-nodulating rhizobia, a group specific DNA probe was also developed, which did not hybridize with more than 20 other common soil and rhizosphere bacteria. Results from several laboratories contributing to knowledge of signal exchange and communication in theRhizobium/Bradyrhizobium legume system are summarized in a new scheme, including also defense reactions at the early stages of legume nodule initiation. Stimulating effects of flavonoids on germination and growth of VA mycorrhiza fungi were also found. A constitutive antifungal compound in pea roots, β-isoxazolinonyl-alanine, was characterized.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-069X
    Keywords: Relapsing polychondritis ; Autoantibody ; Collagen ; Cartilage
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Relapsing polychondritis is a systemic disease associated with a destruction of cartilage in various parts of the body. Sera from six patients with relapsing polychondritis and one patient with microscopic polyarteritis nodosa as well as from six controls were analyzed by immunoblotting and ELISA. All patients had autoantibodies against native collagens II and IX. The serum from one patient showed a strong reaction with all three collagen chains of the high molecular weight fraction of collagen IX after denaturation; sera from four patients showed autoantibodies against α2 (XI) and sera from three patients showed autoantibodies against the covalently cross-linked γ component of collagen XI. The presence of autoantibodies against collagens II, IX, and XI, which form the major fibrillar scaffold in cartilage and mediate the interaction of collagen fibrils and proteoglycan, suggests that autoantibodies against cartilaginous collagen may play a crucial role in the pathogenesis of relapsing polychondritis and microscopic polyarteritis nodosa.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 286 (1994), S. 425-428 
    ISSN: 1432-069X
    Keywords: Ehlers Danlos syndrome ; Collagen
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A patient suffering from a severe form of Ehlers-Danlos syndrome is presented (EDS type VII). The presence of bilateral congenital hip dislocation, generalized joint hypermobility and a soft hyperelastic skin with abnormal scarring suggested a specific collagen type I defect. SDS-PAGE analysis of collagens secreted into the medium of fibroblast cultures showed a retarded migration of more than half of the α2(I) chains. CNBr peptide mapping of the HPLC-purified altered chain localized the mutant locus to the N-terminal region of the protein. cDNA analysis of the corresponding gene COL1A2 revealed, in addition to the expected collagen sequence, a transcript missing the entire exon 6. This exon encodes a major crosslinking site within collagen fibres as well as the N-propeptidase cleavage site. The skipping of exon 6 is caused by a splice site mutation substituting an A for a G at the first nucleotide of intron 6.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1573-5036
    Keywords: Bradyrhizobium ; hopanoids ; nitrogen fixation ; plant-microbe interaction ; Rhizobium ; squalene-hopene cyclase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Agriculture, Forestry, Horticulture, Fishery, Domestic Science, Nutrition
    Notes: Abstract Hopanoid lipids have been discovered recently in a number of nitrogen-fixing soil bacteria and in Bradyrhizobium bacteria which fix nitrogen in association with legume plants. We report here an investigation of the hopanoid content in an additional number of soil bacteria capable of living in close association with plants. Of the strains investigated, hopanoids were discovered in phototrophic, nitrogen-fixing bacteria and in an extended number of Bradyrhizobium strains. Strains in which hopanoids so far have not been found belong to the following genera: Rhizobium, Sinorhizobium, Phyllobacterium, Agrobacterium, and Azoarcus. To address the function of hopanoids in Bradyrhizobium, we cloned the gene coding for a key enzyme of hopanoid biosynthesis, the squalene-hopene cyclase, and expressed the gene in E. coli. The recombinant enzyme catalyzed in vitro the cyclization of squalene to hopanoid derivatives.
    Type of Medium: Electronic Resource
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