IMMUNOLOGICAL EVIDENCE FOR DEFICIENCY IN AN ACTIVATOR PROTEIN FOR SULFATIDE SULFATASE IN A VARIANT FORM OF METACHROMATIC LEUKODYSTROPHY

被引:94
作者
INUI, K
EMMETT, M
WENGER, DA
机构
[1] UNIV COLORADO,HLTH SCI CTR,DEPT PEDIAT,DENVER,CO 80262
[2] UNIV COLORADO,MED CTR,WEBB WARING LUNG INST,DENVER,CO 80262
来源
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES | 1983年 / 80卷 / 10期
关键词
D O I
10.1073/pnas.80.10.3074
中图分类号
Q [生物科学];
学科分类号
07 ; 0710 ; 09 ;
摘要
引用
收藏
页码:3074 / 3077
页数:4
相关论文
共 23 条
[1]   NIEMANN-PICK DISEASE, TYPE-C - EVIDENCE FOR THE DEFICIENCY OF AN ACTIVATING FACTOR STIMULATING SPHINGOMYELIN AND GLUCOCEREBROSIDE DEGRADATION [J].
CHRISTOMANOU, H .
HOPPE-SEYLERS ZEITSCHRIFT FUR PHYSIOLOGISCHE CHEMIE, 1980, 361 (10) :1489-1502
[3]  
EMMETT M, 1981, CLIN IMMUNOL NEWSL, V2, P99
[4]   ACTIVATOR OF CEREBROSIDE-SULFATASE - MODEL OF ACTIVATION [J].
FISCHER, G ;
JATZKEWITZ, H .
BIOCHIMICA ET BIOPHYSICA ACTA, 1978, 528 (01) :69-76
[5]   THE AB-VARIANT OF METACHROMATIC LEUKODYSTROPHY (POSTULATED ACTIVATOR PROTEIN-DEFICIENCY) - LIGHT AND ELECTRON-MICROSCOPIC FINDINGS IN A SURAL NERVE BIOPSY [J].
HAHN, AF ;
GORDON, BA ;
GILBERT, JJ ;
HINTON, GG .
ACTA NEUROPATHOLOGICA, 1981, 55 (04) :281-287
[6]  
HAHN AF, 1981, ANN NEUROL, V12, P33
[7]   PURIFICATION AND PROPERTIES OF HEXOSAMINIDASE A-ACTIVATING PROTEIN FROM HUMAN LIVER [J].
HECHTMAN, P ;
LEBLANC, D .
BIOCHEMICAL JOURNAL, 1977, 167 (03) :693-701
[8]   DEFICIENCY OF THE HEXOSAMINIDASE-A ACTIVATOR PROTEIN IN A CASE OF GM2 GANGLIOSIDOSIS - VARIANT-AB [J].
HECHTMAN, P ;
GORDON, BA ;
KIN, NMKNY .
PEDIATRIC RESEARCH, 1982, 16 (03) :217-222
[9]   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-&
[10]   PROPERTIES OF A PROTEIN ACTIVATOR OF GLYCOSPHINGOLIPID HYDROLYSIS ISOLATED FROM THE LIVER OF A PATIENT WITH GM1 GANGLIOSIDOSIS, TYPE-1 [J].
INUI, K ;
WENGER, DA .
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1982, 105 (02) :745-751