NORMAL N-OLIGOSACCHARYLTRANSFERASE ACTIVITY IN FIBROBLASTS FROM PATIENTS WITH CARBOHYDRATE-DEFICIENT GLYCOPROTEIN SYNDROME

Authors
KNAUER R LEHLE L HANEFELD F VONFIGURA K
Citation
R. Knauer et al., NORMAL N-OLIGOSACCHARYLTRANSFERASE ACTIVITY IN FIBROBLASTS FROM PATIENTS WITH CARBOHYDRATE-DEFICIENT GLYCOPROTEIN SYNDROME, Journal of inherited metabolic disease, 17(5), 1994, pp. 541-544
Citations number
15
Categorie Soggetti
Endocrynology & Metabolism
Journal title
Journal of inherited metabolic disease → ACNP
ISSN journal
01418955
Volume
17
Issue
5
Year of publication
1994
Pages
541 - 544
Database
ISI
SICI code
0141-8955(1994)17:5<541:NNAIFF>2.0.ZU;2-0
Abstract
The N-oligosaccharyltransferase activity was found to be normal in fib roblasts from five patients with the carbohydrate-deficient glycoprote in syndrome type I and type III using an assay based on dolichyldiphos phoryl-N-N'-diacetylchitobiose as donor and a hexapeptide as acceptor substrate.