ACTIVATOR PROTEINS AND TOPOLOGY OF LYSOSOMAL SPHINGOLIPID CATABOLISM

被引：246

作者：

FURST, W ^{[1
]}

SANDHOFF, K ^{[1
]}

机构：

[1] UNIV BONN, INST ORGAN CHEM & BIOCHEM, GERHARD DOMAGK STR 1, W-5300 BONN 1, GERMANY

来源：

BIOCHIMICA ET BIOPHYSICA ACTA | 1992年 / 1126卷 / 01期

关键词：

SPHINGOLIPID ACTIVATOR PROTEIN; GM2-ACTIVATOR; SULFATIDE ACTIVATOR; GLUCOSYLCERAMIDASE ACTIVATOR; SAPOSIN; SPHINGOLIPID; SPHINGOLIPIDOSE; LYSOME;

D O I：

10.1016/0005-2760(92)90210-M

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：

The lysosomal degradation of several sphingolipids by acid hydrolases is dependent on small non-enzymic cofactors, called sphingolipid activator proteins some of which have been identified as sphingolipid binding proteins. This review summarizes the information available on the structure, function, biosynthesis, gene organization and pathobiochemistry of the known sphingolipid activator proteins. It also offers models for their mode of action and for the topology of lysosomal digestion of glycolipids.

引用

页码：1 / 16

页数：16

共 159 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] ACTIVATOR PROTEINS FOR SULFATIDE HYDROLYSIS AND GM1-GANGLIOSIDE HYDROLYSIS - PROBABLE IDENTITY ON THE BASIS OF THEIR COPURIFICATION, PROPERTIES, LIGAND-BINDING AND IMMUNOCHEMICAL INTERACTIONS [J].

BANKS, MN ;

HERBERT, SJ ;

WYNN, CH .

GLYCOCONJUGATE JOURNAL, 1987, 4 (02) :157-170

[3]

BASU A, 1984, J BIOL CHEM, V259, P1714

[4]

BASU A, 1985, J BIOL CHEM, V260, P3067

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

BERENT, SL ;

RADIN, NS .

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

[6] MECHANISM OF ACTIVATION OF GLUCOCEREBROSIDASE BY CO-BETA-GLUCOSIDASE (GLUCOSIDASE ACTIVATOR PROTEIN) [J].

BERENT, SL ;

RADIN, NS .

BIOCHIMICA ET BIOPHYSICA ACTA, 1981, 664 (03) :572-582

[7]

BROWN JR, 1982, LIPID PROTEIN INTERA, V1, P25

[8] ACTIVATING PROTEINS FOR GANGLIOSIDE GM2 DEGRADATION BY BETA-HEXOSAMINIDASE ISOENZYMES IN TISSUE-EXTRACTS FROM DIFFERENT SPECIES [J].

BURG, J ;

BANERJEE, A ;

CONZELMANN, E ;

SANDHOFF, K .

HOPPE-SEYLERS ZEITSCHRIFT FUR PHYSIOLOGISCHE CHEMIE, 1983, 364 (07) :821-829

[9] MOLECULAR-FORMS OF GM2-ACTIVATOR PROTEIN - A STUDY ON ITS BIOSYNTHESIS IN HUMAN-SKIN FIBROBLASTS [J].

BURG, J ;

BANERJEE, A ;

SANDHOFF, K .

BIOLOGICAL CHEMISTRY HOPPE-SEYLER, 1985, 366 (09) :887-891

[10] MAPPING OF THE GENE CODING FOR THE HUMAN GM2 ACTIVATOR PROTEIN TO CHROMOSOME-5 [J].

BURG, J ;

CONZELMANN, E ;

SANDHOFF, K ;

SOLOMON, E ;

SWALLOW, DM .

ANNALS OF HUMAN GENETICS, 1985, 49 (JAN) :41-45

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