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

被引：18

作者：

KNAUER, R

LEHLE, L

HANEFELD, F

VONFIGURA, K

机构：

[1] UNIV GOTTINGEN, KINDERHEILKUNDE ABT, D-37075 GOTTINGEN, GERMANY

[2] UNIV GOTTINGEN, BIOCHEM ABT 2, D-37073 GOTTINGEN, GERMANY

来源：

JOURNAL OF INHERITED METABOLIC DISEASE | 1994年 / 17卷 / 05期

关键词：

D O I：

10.1007/BF00711588

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：541 / 544

页数：4

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