SOLUBILIZATION OF GLUCOCEREBROSIDASE FROM HUMAN PLACENTA AND DEMONSTRATION OF A PHOSPHOLIPID REQUIREMENT FOR ITS CATALYTIC ACTIVITY

被引:60
作者
DALE, GL [1 ]
VILLACORTE, DG [1 ]
BEUTLER, E [1 ]
机构
[1] CITY HOPE NATL MED CTR, DIV MED, DUARTE, CA 91010 USA
关键词
D O I
10.1016/0006-291X(76)90760-9
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
Glucocerebrosidase [human placenta], a membrane bound enzyme, can be solubilized by acetone precipitation, and the resultant soluble enzyme activity can be demonstrated in the presence of acidic phospholipid, e.g., phosphatidylserine. This is the 1st report of the detergent-free solubilization of glucocerebrosidase, which, when defective, results in Gaucher''s disease.
引用
收藏
页码:1048 / 1053
页数:6
相关论文
共 10 条
[1]  
BEUTLER E, 1970, J LAB CLIN MED, V76, P747
[2]  
BEUTLER E, 1970, LANCET, V1, P612
[3]   METABOLISM OF GLUCOCEREBROSIDES .2. EVIDENCE OF AN ENZYMATIC DEFICIENCY IN GAUCHERS DISEASE [J].
BRADY, RO ;
KANFER, JN ;
SHAPIRO, D .
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1965, 18 (02) :221-&
[4]   TOWARD ENZYME THERAPY FOR LYSOSOMAL STORAGE DISEASES [J].
DESNICK, RJ ;
THORPE, SR ;
FIDDLER, MB .
PHYSIOLOGICAL REVIEWS, 1976, 56 (01) :57-99
[5]   GAUCHERS DISEASE - DEFICIENCY OF ACID BETA-GLUCOSIDASE AND RECONSTITUTION OF ENZYME ACTIVITY IN-VITRO [J].
HO, MW ;
OBRIEN, JS .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1971, 68 (11) :2810-&
[6]  
HO MW, 1973, BIOCHEM J, V136, P821, DOI 10.1042/bj1360821
[7]   BETA-GLUCOSIDASE ACTIVITIES IN HUMAN TISSUES FINDINGS IN GAUCHERS DISEASE [J].
HULTBERG, B ;
OCKERMAN, PA .
CLINICA CHIMICA ACTA, 1970, 28 (01) :169-&
[8]   A DEFICIENCY OF GLUCOCEREBROSIDASE IN GAUCHERS DISEASE [J].
PATRICK, AD .
BIOCHEMICAL JOURNAL, 1965, 97 (02) :C17-&
[9]  
PENTCHEV PG, 1973, J BIOL CHEM, V248, P5256
[10]  
ROSENBERG A, 1958, J BIOL CHEM, V233, P1323