1. [Glycolipids of the cell surface--biochemistry of their decomposition].
- Author
-
Sandhoff K and Kolter T
- Subjects
- Animals, Carbohydrate Conformation, Carbohydrate Sequence, Cell Membrane ultrastructure, Endocytosis, Eukaryotic Cells, Glycolipids biosynthesis, Glycolipids chemistry, Glycosphingolipids biosynthesis, Glycosphingolipids chemistry, Humans, Lysosomal Storage Diseases metabolism, Lysosomes metabolism, Membrane Lipids biosynthesis, Membrane Lipids chemistry, Models, Structural, Molecular Sequence Data, Cell Membrane metabolism, Glycolipids metabolism, Glycosphingolipids metabolism, Membrane Lipids metabolism
- 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.
- Published
- 1995