Abstract |
Glycosphingolipids (GSL) form cell-type-specific patterns on the surface of eukaryotic cells. For the maintenance of these patterns, biosynthesis, intracellular transport, and degradation of GSL are thought to proceed in a coordinated fashion. After transport from the plasma membrane to the lysosomes the degradation of GSL requires protein cofactors to solubilize the membrane-bound substrates of the degradative enzymes. Inborn errors of metabolism leading to defective hydrolases or activator proteins give rise to sphingolipid storage diseases; in some cases the residual enzyme activity can be correlated with the clinical manifestations.
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Authors | K Sandhoff, T Kolter |
Journal | Die Naturwissenschaften
(Naturwissenschaften)
Vol. 82
Issue 9
Pg. 403-13
(Sep 1995)
ISSN: 0028-1042 [Print] Germany |
Vernacular Title | Glykolipide der Zelloberfläche--Biochemie ihres Abbaus. |
PMID | 7477413
(Publication Type: English Abstract, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Glycolipids
- Glycosphingolipids
- Membrane Lipids
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Topics |
- Animals
- Carbohydrate Conformation
- Carbohydrate Sequence
- Cell Membrane
(metabolism, ultrastructure)
- Endocytosis
- Eukaryotic Cells
- Glycolipids
(biosynthesis, chemistry, metabolism)
- Glycosphingolipids
(biosynthesis, chemistry, metabolism)
- Humans
- Lysosomal Storage Diseases
(metabolism)
- Lysosomes
(metabolism)
- Membrane Lipids
(biosynthesis, chemistry, metabolism)
- Models, Structural
- Molecular Sequence Data
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