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

页码：3074 / 3077

页数：4

共 23 条

[1] NIEMANN-PICK DISEASE, TYPE-C - EVIDENCE FOR THE DEFICIENCY OF AN ACTIVATING FACTOR STIMULATING SPHINGOMYELIN AND GLUCOCEREBROSIDE DEGRADATION
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[J]. HOPPE-SEYLERS ZEITSCHRIFT FUR PHYSIOLOGISCHE CHEMIE, 1980, 361 (10): : 1489 - 1502
[2] AB-VARIANT OF INFANTILE GM2 GANGLIOSIDOSIS - DEFICIENCY OF A FACTOR NECESSARY FOR STIMULATION OF HEXOSAMINIDASE A-CATALYZED DEGRADATION OF GANGLIOSIDE GM2 AND GLYCOLIPID GA2
CONZELMANN, E
SANDHOFF, K
[J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1978, 75 (08) : 3979 - 3983
[3] EMMETT M, 1981, CLIN IMMUNOL NEWSL, V2, P99
[4] ACTIVATOR OF CEREBROSIDE-SULFATASE - MODEL OF ACTIVATION
FISCHER, G
JATZKEWITZ, H
[J]. 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
HAHN, AF
GORDON, BA
GILBERT, JJ
HINTON, GG
[J]. 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
HECHTMAN, P
LEBLANC, D
[J]. BIOCHEMICAL JOURNAL, 1977, 167 (03) : 693 - 701
[8] DEFICIENCY OF THE HEXOSAMINIDASE-A ACTIVATOR PROTEIN IN A CASE OF GM2 GANGLIOSIDOSIS - VARIANT-AB
HECHTMAN, P
GORDON, BA
KIN, NMKNY
[J]. PEDIATRIC RESEARCH, 1982, 16 (03) : 217 - 222
[9] GAUCHERS DISEASE - DEFICIENCY OF ACID BETA-GLUCOSIDASE AND RECONSTITUTION OF ENZYME ACTIVITY IN-VITRO
HO, MW
OBRIEN, JS
[J]. 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
INUI, K
WENGER, DA
[J]. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1982, 105 (02) : 745 - 751

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