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.
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页码：1097 / 1103
页数：7
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