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  • 1
    Electronic Resource
    Electronic Resource
    A patient with features of both Bardet-Biedl and Alström syndromes (1990)
    Springer
    European journal of pediatrics 149 (1990), S. 783-785 
    Details
    ISSN: 1432-1076
    Keywords: Alström syndrome ; Bardet-Biedl syndrome ; Laurence-Moon syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe a 30-year-old patient with acanthosis nigricans, diabetes mellitus with insulin resistance, hypogonadotropic hypogonadism, pigmentary degeneration of the retina and cerebellar, pyramidal and posteri-or columnar involvement. He had normal mental function, normal hearing and no hexadactyly. The patient had symptoms of both Bardet-Biedl and Alström syndromes, but did not manifest all the main features of either syndrome. This suggests either that the Bardet-Biedl, Alström, Laurence-Moon syndromes (including the variant described by Edwards) have a highly variable expression or that our case is a new variant within this group of syndromes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957281
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Granulocyte-activating mediators (GRAM) (1987)
    Springer
    Archives of dermatological research 279 (1987), S. 470-477 
    Details
    ISSN: 1432-069X
    Keywords: Granulocyte-activating ; mediators (GRAM) ; Epidermoid carcinoma ; Lipopoly-saccharide ; Cytokines ; Langerhans cells ; Epidermal cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In the present study we investigated the capability of human epidermal cells to generate granulocyte-activating mediators (GRAM). It could be shown that human epidermal cells as well as an epidermoid carcinoma cell line (A431) produce an epidermal cell-derived granulocyte-activating mediator (EC-GRAM) which stimulates human granulocytes to release significant levels of toxic oxygen radicals as measured by a lucigenin-dependent chemiluminescence (CL). For further characterization of EC-GRAM the A431 cell line was used. Supernatants of A431 cells usually contained maximal EC-GRAM levels within 24 h of incubation. Factor production was enhanced by bacterial lipopolysaccharide (LPS), but not by silica particles and PHA. Moreover, freeze-thaw lysates of A431 cells and extracts of heat-separated human epidermis contained significant levels of EC-GRAM. Preincubation of granulocytes with EC-GRAM resulted in an enhanced response to subsequent stimulation with the chemotactic peptide f-met-phe. In contrast EC-GRAM did not affect the response to PMA or zymosan particles. However, EC-GRAM treated granulocytes were unresponsive to restimulation with EC-GRAM. Upon high performance liquid chromatography (HPLC) gel filtration EC-GRAM eluted within two major peaks exhibiting a molecular weight of 17 kD and 44 kD. According to its biochemical and biological properties EC-GRAM can be separated from other cytokines such as ETAF/-interleukin 1, interleukin 2, interferons, granulocyte colony-stimulating factor (G-CSF) and tumor necrosis factor (TNF). However, an antibody to human GM-CSF neutralized about 75% of the activity. These results indicate that EC-GRAM activity stimulating the generation of reactive oxygen species by granulocytes is probably due to GM-CSF.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00412594
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    Paper (German National Licenses)
    Fulltext
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