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  • Title: Biosynthesis of acid alpha-glucosidase in late-onset forms of glycogenosis type II (Pompe's disease).
    Author: Steckel F, Gieselmann V, Waheed A, Hasilik A, von Figura K, Oude Elferink R, Kalsbeek R, Tager JM.
    Journal: FEBS Lett; 1982 Dec 13; 150(1):69-76. PubMed ID: 6761145.
    Abstract:
    Cultured human skin fibroblasts from control persons and from patients with the generalized and late-onset forms of Pompe's disease were labelled with radioactive leucine and the incorporation of radioactivity into acid alpha-glucosidase and cathepsin D was analysed by immunoprecipitation, gel electrophoresis and fluorography. When the labelling was carried out for 6-12 h in the presence of NH4Cl, the labelling of secreted alpha-glucosidase relative to that of secreted cathepsin D in fibroblasts from patients with the late-onset form of Pompe's disease was less than 15% of that in fibroblasts from control persons. However, when the fibroblasts were labelled for less than 1 h, the relative rate of incorporation of radioactivity into acid alpha-glucosidase was rather similar in the two types of fibroblasts. In fibroblasts from patients with the generalized form of Pompe's disease no incorporation of radioactivity into acid alpha-glucosidase could be detected.
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