STUDIES ON A SPHINGOLIPID ACTIVATOR PROTEIN (SAP-2) IN FIBROBLASTS FROM PATIENTS WITH LYSOSOMAL STORAGE DISEASES, INCLUDING NIEMANN-PICK DISEASE TYPE-C

被引：31

作者：

FUJIBAYASHI, S ^{[1
]}

WENGER, DA ^{[1
]}

机构：

[1] UNIV COLORADO,SCH MED,DEPT PEDIAT,DENVER,CO 80262

来源：

CLINICA CHIMICA ACTA | 1985年 / 146卷 / 2-3期

关键词：

D O I：

10.1016/0009-8981(85)90053-1

中图分类号：

R446 [实验室诊断]; R-33 [实验医学、医学实验];

学科分类号：

1001 ;

摘要：

引用

页码：147 / 156

页数：10

共 24 条

[1] ENZYME-LINKED IMMUNOSORBENT-ASSAY FOR THE GANGLIOSIDE GM2-ACTIVATOR PROTEIN - SCREENING OF NORMAL HUMAN-TISSUES AND BODY-FLUIDS, OF TISSUES OF GM2 GANGLIOSIDOSIS, AND FOR ITS SUBCELLULAR-LOCALIZATION [J].

BANERJEE, A ;

BURG, J ;

CONZELMANN, E ;

CARROLL, M ;

SANDHOFF, K .

HOPPE-SEYLERS ZEITSCHRIFT FUR PHYSIOLOGISCHE CHEMIE, 1984, 365 (03) :347-356

[2] ACTIVATORS FOR SPHINGOHYDROLASES AND NATURE OF SPHINGOMYELINASE DEFICIENCY IN NIEMANN-PICK DISEASE TYPES A, B AND C [J].

BARATON, G ;

REVOL, A .

CLINICA CHIMICA ACTA, 1977, 76 (03) :339-343

[3] BETA-GLUCOSIDASE ACTIVATOR PROTEIN FROM BOVINE SPLEEN (COGLUCOSIDASE) [J].

BERENT, SL ;

RADIN, NS .

ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS, 1981, 208 (01) :248-260

[4] 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

[5] AB-VARIANT OF INFANTILE GM2 GANGLIOSIDOSIS - DEFICIENCY OF A FACTOR NECESSARY FOR STIMULATION OF HEXOSAMINIDASE A-CATALYZED DEGRADATION OF GANGLIOSIDE GM2 AND GLYCOLIPID GA2 [J].

CONZELMANN, E ;

SANDHOFF, K .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1978, 75 (08) :3979-3983

[6]

EMMETT M, 1981, CLIN IMMUNOL NEWSL, V2, P99

[7] ACTIVATOR PROTEIN-DEFICIENT METACHROMATIC LEUKODYSTROPHY - DIAGNOSIS IN LEUKOCYTES USING IMMUNOLOGICAL METHODS [J].

FUJIBAYASHI, S ;

INUI, K ;

WENGER, DA .

JOURNAL OF PEDIATRICS, 1984, 104 (05) :739-742

[8] THE PROTEIN ACTIVATOR SPECIFIC FOR THE ENZYMIC-HYDROLYSIS OF GM2 GANGLIOSIDE IN NORMAL HUMAN-BRAIN AND BRAINS OF 3 TYPES OF GM2 GANGLIOSIDOSIS [J].

HIRABAYASHI, Y ;

LI, YT ;

LI, SC .

JOURNAL OF NEUROCHEMISTRY, 1983, 40 (01) :168-175

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

INUI, K ;

EMMETT, M ;

WENGER, DA .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1983, 80 (10) :3074-3077

← 1 2 3 →