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NormalN-oligosaccharyltransferase activity in fibroblasts from patients with carbohydrate-deficient glycoprotein syndrome

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Journal of Inherited Metabolic Disease

Summary

TheN-oligosaccharyltransferase activity was found to be normal in fibroblasts from five patients with the carbohydrate-deficient glycoprotein syndrome type I and type III using an assay based on dolichyldiphosphoryl-N-N′-diacetylchitobiose as donor and a hexapeptide as acceptor substrate.

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Authors and Affiliations

  1. Universität Regensburg, Lehrstuhl für Zellbiologie und Pflanzenphysiologie, Universitätsstraβe, D-93053, Regensburg

    R. Knauer & L. Lehle

  2. Georg-August-Universität Göttingen, Abt. Kinderheilkunde, Schwerpunkt Neuropaediatrie, Robert-Koch-Str. 40, D-37075, Göttingen

    F. Hanefeld

  3. Georg-August-Universität Göttingen, Abt. Biochemie II, Gosslerstr. 12d, D-37073, Göttingen, Germany

    K. von Figura

Authors
  1. R. Knauer
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  2. L. Lehle
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  3. F. Hanefeld
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  4. K. von Figura
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Knauer, R., Lehle, L., Hanefeld, F. et al. NormalN-oligosaccharyltransferase activity in fibroblasts from patients with carbohydrate-deficient glycoprotein syndrome. J Inherit Metab Dis 17, 541–544 (1994). https://doi.org/10.1007/BF00711588

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  • DOI: https://doi.org/10.1007/BF00711588

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