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[Glycolipids of the cell surface--biochemistry of their decomposition].

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.
AuthorsK Sandhoff, T Kolter
JournalDie Naturwissenschaften (Naturwissenschaften) Vol. 82 Issue 9 Pg. 403-13 (Sep 1995) ISSN: 0028-1042 [Print] Germany
Vernacular TitleGlykolipide der Zelloberfläche--Biochemie ihres Abbaus.
PMID7477413 (Publication Type: English Abstract, Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Glycolipids
  • Glycosphingolipids
  • Membrane Lipids
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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