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  • 2000-2004  (2)
  • cystic fibrosis  (2)
  • Analytical Chemistry and Spectroscopy
  • Inorganic Chemistry
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Glycoconjugate journal 17 (2000), S. 617-626 
    ISSN: 1573-4986
    Keywords: α1,3fucose ; α1,6fucose ; sialic acid ; polyα2,8sialic acid ; surface membrane glycoforms ; cystic fibrosis ; lactosylated polylysine gene therapy vector
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Notes: Abstract Terminal glycosylation has been a recurring theme of the laboratory. In cystic fibrosis (CF), decreased sialic acid and increased fucosyl residues in α1,3 position to antennary N -acetyl glucosamine is the CF glycosylation phenotype. The glycosylation phenotype is reversed by transfection of CF airway cells with wtCFTR. In neuronal cells, polymers of α2,8sialyl residues are prominent in oligodendrocytes and human neuroblastoma. These findings are discussed in relationship to early studies in our laboratories and those of other investigators. The potential extension of these concepts to future clinical therapeutics is presented.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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  • 2
    ISSN: 1573-4986
    Keywords: glycosylation ; cystic fibrosis ; airway epithelial cells ; α1,3fucose ; sialic acid ; CFTR ; CFTR transfection
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Notes: Abstract Cystic fibrosis (CF) has a characteristic glycosylation phenotype usually expressed as a decreased ratio of sialic acid to fucose. The glycosylation phenotype was found in CF/T1 airway epithelial cells (ΔF508/ΔF508). When these cells were transfected and were expressing high amounts of wtCFTR, as detected by Western blot analysis and in situ hybridization, the cell membrane glycoconjugates had an increased sialic acid content and decreased fucosyl residues in α1,3/4 linkage to antennary N[emsp4 ]-acetyl glucosamine (Fucα1,3/4GlcNAc). After the expression of wtCFTR decreased, the amount of sialic acid and Fucα1,3/4GlcNAc returned to levels shown by the parent CF cells. Sialic acid was measured by chemical analysis and Fucα1,3/4GlcNAc was detected with a specific α1,3/4 fucosidase. CF and non-CF airway cells in primary culture also had a similar reciprocal relationship between fucosylation and sialylation. It is possible that the glycosylation phenotype is involved in the pathogenesis of CF lung disease by facilitating bacterial colonization and leukocyte recruitment.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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