BIOSYNTHESIS OF HUMAN ALPHA-N-ACETYLGALACTOSAMINIDASE - DEFECTIVE PHOSPHORYLATION AND MATURATION IN INFANTILE ALPHA-NAGA DEFICIENCY

被引：10

作者：

HU, P

REUSER, AJJ

JANSE, HC

KLEIJER, WJ

SCHINDLER, D

SAKURABA, H

TSUJI, A

SUZUKI, Y

VANDIGGELEN, OP

机构：

[1] ERASMUS UNIV,DEPT CLIN GENET,POB 1738,3000 DR ROTTERDAM,NETHERLANDS

[2] ERASMUS UNIV,DEPT CELL BIOL & GENET,3000 DR ROTTERDAM,NETHERLANDS

[3] UNIV WURZBURG,DEPT HUMAN GENET,W-8700 WURZBURG,GERMANY

[4] TOKYO METROPOLITAN INST MED SCI,DEPT CLIN GENET,TOKYO 113,JAPAN

[5] UNIV TOKUSHIMA,DEPT BIOL SCI & TECHNOL,TOKUSHIMA 770,JAPAN

来源：

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS | 1991年 / 175卷 / 03期

关键词：

D O I：

10.1016/0006-291X(91)91678-6

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

The biosynthesis of human α-N-acetylgalactosaminidase (α-NAGA) was studied in normal fibroblasts and in cells from patients with infantile α-NAGA deficiency. Normal α-NAGA is synthesized as a 52 kDa precursor which matures to a 49 kDa species through phosphorylation and carbohydrate trimming. Fibroblasts from the patients synthesize normal amounts of a 52 kDa precursor, however phosphorylation does not occur and this precursor is subsequently degraded intracellularly. © 1991.

引用

页码：1097 / 1103

页数：7

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