Biochemistry of glycosphingolipid degradation.
Glycosphingolipids (GSLs) form cell-type-specific patterns on the surface of eukaryotic cells. Degradation of GSLs requires endocytotic membrane flow of plasma membrane-derived GSLs into the lysosomes as the digesting organelles. Recent research focused on the mechanisms leading to selective membrane degradation in the lysosomes and on the mechanism and physiological function of sphingolipid activator proteins, which are needed for degradation of GSLs with short oligosaccharide chains in addition to hydrolysing enzymes. Both, the inherited deficiency of lysosomal hydrolases and of sphingolipid activator proteins give rise to sphingolipid storage diseases. In some cases it was possible to correlate residual enzyme activities with the onset and the course of the disease.
Sandhoff
K
K
Kekulé-Institut für Organische Chemie und Biochemie der Universität, Bonn, Germany. sandhoff@uni-bonn.de
Kolter
T
T
D000818:Animals; D004195:Disease Models, Animal; D004705:Endocytosis; D049289:G(M2) Activator Protein; D005678:G(M2) Ganglioside / Q000378:metabolism; D006028:Glycosphingolipids / Q000378:metabolism*; D006801:Humans; D006868:Hydrolysis; D008247:Lysosomes / Q000378:metabolism; D011506:Proteins / Q000378:metabolism; D013661:Tay-Sachs Disease / Q000378:metabolism
D049289:G(M2) Activator Protein; D006028:Glycosphingolipids; D011506:Proteins; D005678:G(M2) Ganglioside