ACTIVATION OF LYSOSOMAL HYDROLYSIS OF COMPLEX GLYCOLIPIDS BY NON-ENZYMIC PROTEINS

被引：27

作者：

SANDHOFF, K

CONZELMANN, E

机构：

[1] K. Sandhoff and E. Conzelmann are, the Max-Planck-Institut für Psychiatrie

来源：

TRENDS IN BIOCHEMICAL SCIENCES | 1979年 / 4卷 / 10期

关键词：

D O I：

10.1016/0968-0004(79)90125-7

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：

A large variety ofglycosphingolipids is found at the surface ofanimal cells. Breakdown of these lipids occurs in lysosomes. The interaction of lysosomal hydrolases and amphiphilic lipids is mediated by non-enzymic activator proteins. Deficiencies o f hydrolases or activator proteins result in fatal lipid storage diseases. © 1979.

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页码：231 / 233

页数：3

相关论文

共 24 条

[1] 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

[2] PURIFICATION, BIOCHEMICAL AND IMMUNOLOGICAL CHARACTERIZATION OF HEXOSAMINIDASE-A FROM VARIANT AB OF INFANTILE GM2 GANGLIOSIDOSIS [J].

CONZELMANN, E ;

SANDHOFF, K ;

NEHRKORN, H ;

GEIGER, B ;

ARNON, R .

EUROPEAN JOURNAL OF BIOCHEMISTRY, 1978, 84 (01) :27-33

[3]

CONZELMANN E, 1979, STRUCTURE FUNCTION G

[4] ACTIVATOR OF CEREBROSIDE-SULFATASE - MODEL OF ACTIVATION [J].

FISCHER, G ;

JATZKEWITZ, H .

BIOCHIMICA ET BIOPHYSICA ACTA, 1978, 528 (01) :69-76

[5] ENZYMES OF COMPLEX LIPID-METABOLISM [J].

GATT, S ;

BARENHOLZ, Y .

ANNUAL REVIEW OF BIOCHEMISTRY, 1973, 42 :61-90

[6]

GEIGER B, 1976, BIOCHEMISTRY, V15, P311

[7] STRUCTURES AND ORGANIZATION OF CELL-SURFACE GLYCOLIPIDS DEPENDENCY ON CELL-GROWTH AND MALIGNANT TRANSFORMATION [J].

HAKOMORI, SI .

BIOCHIMICA ET BIOPHYSICA ACTA, 1975, 417 (01) :55-89

[8] ULTRASTRUCTURAL LOCALIZATION OF CELL-MEMBRANE GM1 GANGLIOSIDE BY CHOLERA TOXIN - (IMMUNOELECTRON MICROSCOPY MEMBRANE RECEPTOR VIBRIO-CHOLERAE SIALIDASE GANGLIOSIDE TITRATION) [J].

HANSSON, HA ;

HOLMGREN, J ;

SVENNERHOLM, L .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1977, 74 (09) :3782-3786

[9] PURIFICATION AND PROPERTIES OF HEXOSAMINIDASE A-ACTIVATING PROTEIN FROM HUMAN LIVER [J].

HECHTMAN, P ;

LEBLANC, D .

BIOCHEMICAL JOURNAL, 1977, 167 (03) :693-701

[10] 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-&