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The FASEB Journal, Vol 5, 301-308, Copyright © 1991 by The Federation of American Societies for Experimental Biology

REVIEWS

Saposin proteins: structure, function, and role in human lysosomal storage disorders

JS O'Brien and Y Kishimoto
Department of Neurosciences, University of California, San Diego, La Jolla 92093.

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.


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Copyright © 1991 by The Federation of American Societies for Experimental Biology.