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

FASEB J. 1991 Mar 1;5(3):301-8. doi: 10.1096/fasebj.5.3.2001789.


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.

Publication types

  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Amino Acid Sequence
  • Glycoproteins / chemistry
  • Glycoproteins / physiology*
  • Humans
  • Metabolism, Inborn Errors / etiology*
  • Molecular Sequence Data
  • Protein Conformation
  • Proteins / chemistry
  • Proteins / physiology*
  • Saposins
  • Sphingolipid Activator Proteins


  • Glycoproteins
  • PSAP protein, human
  • Proteins
  • Saposins
  • Sphingolipid Activator Proteins