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THE PRO-PEPTIDE OF THE PRO-BETA-POLYPEPTIDE CHAIN OF HUMAN BETA-HEXOSAMINIDASE IS NECESSARY FOR PROPER PROTEIN-FOLDING AND EXIT FROM THE ENDOPLASMIC-RETICULUM

被引：8
作者：
SAGHERIAN, C
THORNER, P
MAHURAN, D
机构：
[1] HOSP SICK CHILDREN,RES INST,TORONTO M5G 1X8,ON,CANADA
[2] UNIV TORONTO,DEPT CLIN BIOCHEM,TORONTO,ON,CANADA
[3] UNIV TORONTO,DEPT PATHOL,TORONTO,ON,CANADA
来源：
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS | 1994年 / 204卷 / 01期
关键词：
D O I：
10.1006/bbrc.1994.2436
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：
We examine the function of the pro beta-peptide (residues 42-121) in the folding and intracellular transport of human beta-hexosaminidase B (beta-N-acetylhexosaminidase, EC 3.2.1.52). A construct was prepared that encoded an in frame deletion of residues 55-118. Expression of this construct in COS-1 cells produced a beta-polypeptide chain that formed insoluble aggregates and remained trapped in the endoplasmic reticulum (ER). We conclude that the pro beta-peptide may act as a type of intramolecular chaperone for the mature beta-subunit. (C) 1994 Academic Press, Inc.
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收藏
页码：135 / 141
页数：7
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