Saposin proteins: structure, function, and role in human lysosomal storage disorders.
Saposins are sphingolipid activator proteins, four of which are derived from a single precursor, prosaposin, by proteolytic processing. These small heat-stable glycoproteins (12-14 kDa) are required for the lysosomal hydrolysis of a variety of sphingolipids. Characterization of these four activator proteins, two of which were recently discovered, and their importance in human health and disease are reviewed in this article.
O'Brien
J S
JS
Department of Neurosciences, University of California, San Diego, La Jolla 92093.
Kishimoto
Y
Y
D000595:Amino Acid Sequence; D006023:Glycoproteins / Q000737:chemistry / Q000502:physiology*; D006801:Humans; D008661:Metabolism, Inborn Errors / Q000209:etiology*; D008969:Molecular Sequence Data; D011487:Protein Conformation; D011506:Proteins / Q000737:chemistry / Q000502:physiology*; D049231:Saposins; D049128:Sphingolipid Activator Proteins
D006023:Glycoproteins; C483345:PSAP protein, human; D011506:Proteins; D049231:Saposins; D049128:Sphingolipid Activator Proteins