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  • Electronic Resource  (3)
  • Glycogen storage disease type Ib  (2)
  • Glycoprotein microheterogeneity  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Impaired metabolic function of polymorphonuclear leukocytes in glycogen storage disease Ib (1983)
    Springer
    European journal of pediatrics 140 (1983), S. 329-330 
    Details
    ISSN: 1432-1076
    Keywords: Glycogen storage disease type Ib ; Polymorphonuclear leukocyte function ; Bactericidal activity ; Superoxide production ; Hexose monophosphate shunt
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To elucidate the basis for the recurrent infections in patients with glycogen storage disease (GSD) Ib we tested polymorphonuclear leukocyte (PMN) function in one patient. Bactericidal capacity and phagocytosis-induced O2 consumption were reduced. Also, phorbol myristate acetate-stimulated superoxide production and glucose oxidation through the hexose monophosphate shunt were diminished compared to control subjects. Therefore it could be speculated that in PMN of patients with GSD Ib, glucose-6-phosphate has no access to the enzymes of the hexose monophosphate shunt due to a transport-related defect as shown for glucogenesis in hepatocytes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442674
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Glycogen storage disease type Ib: familial bleeding tendency (1984)
    Springer
    European journal of pediatrics 143 (1984), S. 7-9 
    Details
    ISSN: 1432-1076
    Keywords: Glycogen storage disease type Ib ; Familial bleeding tendency ; Platelet dysfunction ; von Willebrand disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A mild bleeding tendency with characteristics of the von Willebrand disease was documented in family members of a girl with glycogen storage disease type Ib (GSD) Ib). It was assumed that a defective glucose-6-phosphate dependent microsomal glycoprotein synthesis was involved in the bleeding disorder of the patient and the GSD Ib heterozygotes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442739
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    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    Glycogen storage disease Ib: modification of α1 glycoprotein microheterogeneity (1989)
    Springer
    European journal of pediatrics 148 (1989), S. 341-343 
    Details
    ISSN: 1432-1076
    Keywords: Glycogen storage disease Ib ; α1-Antitrypsin ; Glycoprotein synthesis ; Glycoprotein microheterogeneity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Upon isoelectric focusing, α1-antitrypsin shows a characteristic major band pattern caused by glycoprotein isoforms with different oligosaccharide side chains. In ten apparently healthy individuals with the Pi type MM the ratio of isoforms I, II, and III was 5.2:3.9:1.0. In six patients with glycogen storage disease Ib the ratio of isoforms in the Pi type MM was 3.6:5.5:1.9 suggesting a shift from the biantennary to triantennary oligosaccharide side chains. This shift may result from the limited availability of glucose or glucose derivatives for the synthesis of N-glycosidic glycoproteins.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00444130
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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