Biosynthesis of acid alpha-glucosidase in late-onset forms of glycogenosis type II (Pompe's disease) |
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Authors: | F Steckel V Gieselmann A Waheed A Hasilik K von Figura R Oude Elferink R Kalsbeek J M Tager |
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Institution: | 1. Physiologisch-Chemisches Institut, Westfälische Wilhelms-Universität, Waldeyerstrasse 15, 44 Münster (Westf.), FRG;2. Laboratorium voor Biochemie, Universiteit van Amsterdam, Plantage Muidergracht 12, 1018 TV Amsterdam, The Netherlands |
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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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Keywords: | Acid α-glucosidase Pompe's disease Lysosomal α-glucosidase Glycogenosis type II Lysosomal enzyme synthesis |
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