Review
doi: 10.1096/fasebj.5.3.2001789.
Saposin proteins: structure, function, and role in human lysosomal storage disorders
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- PMID: 2001789
- DOI: 10.1096/fasebj.5.3.2001789
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Review
Saposin proteins: structure, function, and role in human lysosomal storage disorders
FASEB J.
.
Abstract
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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