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Normal N ‐oligosaccharyltransferase activity in fibroblasts from patients with carbohydrate‐deficient glycoprotein syndrome
Author(s) -
Knauer R.,
Lehle L.,
Hanefeld F.,
Figura K.
Publication year - 1994
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf00711588
Subject(s) - glycoprotein , carbohydrate , biochemistry , chemistry , microbiology and biotechnology , biology
Summary The N ‐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.