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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 59 (1981), S. 91-93 
    ISSN: 1432-1440
    Keywords: Collagen synthesis ; Fibroblast cultures ; Osteogenesis imperfecta ; Kollagensynthese ; Fibroblastenkulturen ; Osteogenesis imperfecta
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Hautfibroblasten wurden von 13 Patienten mit Osteogenesis imperfecta gezüchtet und deren Kollagensynthese in vitro untersucht. Dabei fand sich bei 7 Patienten, die durch nur milde Manifestation der Erkrankung charakterisiert waren, eine Störung des Verhältnisses der Kollagentypen I und III. Fibroblasten von solchen Patienten mit einer schweren Form der Osteogenesis imperfecta synthetisierten die Kollagentypen I und III in einem normalen Verhältnis.
    Notes: Summary Skin fibroblasts were cultured from 13 patients with Osteogenesis imperfecta and collagen biosynthesis was investigated in vitro. In those patients characterised by only mild manifestations of the disease, the ratio of collagen types I and III was disturbed. By contrast, fibroblasts obtained from patients with Osteogenesis imperfecta of a more severe type synthesised collagen types I/III in a normal ratio.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 124 (1977), S. 101-111 
    ISSN: 1432-1076
    Keywords: Hutchinson-Gilford-syndrome ; Familial incidence ; Autosomal-recessive disorder ; Fibroblast cultures ; Collagen types ; Thymidine incorporation ; Immunofluorescence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report relates the case histories of two sisters who demonstrated the typical symptoms of progeria at birth. One of these children had died previous to this study. The familial occurrence underlines the thesis that progeria is an autosomal-recessive disorder. The examination of the cultured skin fibroblasts from the younger child showed a clear decrease in cell growth. On the other hand, the immunfluorescent examination of skin biopsies and cultured skin fibroblasts revealed no atypical distribution of collagen types.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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