Distribution of saposins (sphingolipid activator proteins) in tissues of lysosomal storage disease patients.

Abstract

Saposins are a group of small glycoproteins derived from a single precursor protein, prosaposin. Each of the four saposins are involved in lysosomal hydrolysis of various sphingolipids. Our recent investigations have shown that saposins accumulate in tissues of several lysosomal storage diseases patients, including those with Tay-Sachs disease and Gaucher disease. To obtain insight into the mechanism of accumulation and its pathological role, the subcellular distribution of saposins in brain from Tay-Sachs disease and in spleen of Gaucher disease were compared with that of GM2 ganglioside and glucocerebroside, respectively. In both Tay-Sachs brain and Gaucher spleen, saposins were found predominantly in light-density fractions while most of the GM2 ganglioside and glucocerebroside, respectively, were found in heavy-density fractions. These studies indicate that saposins that accumulate in these pathological tissues are not tightly associated with GM2 ganglioside or glucocerebroside.

• Authors: M Tayama, J S O'Brien, Y Kishimoto
• Journal: Journal of molecular neuroscience : MN (J Mol Neurosci) Vol. 3 Issue 4 Pg. 171-5 ( 1992) ISSN: 0895-8696 [Print] United States
• PMID: 1389998 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

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

Topics

• Brain (pathology)
• Brain Chemistry
• Gaucher Disease (metabolism, pathology)
• Glycoproteins (analysis)
• Humans
• Saposins
• Sphingolipid Activator Proteins
• Spleen (chemistry, pathology)
• Subcellular Fractions (chemistry)
• Tay-Sachs Disease (metabolism, pathology)