Immunological studies on sphingolipid activator proteins in the neuronal ceroid-lipofuscinoses.

Abstract

The neuronal ceroid-lipofuscinoses constitute an important group of progressive encephalopathies leading to severe psychomotor retardation, blindness, and early death. They are characterized by accumulation of autofluorescent, electron-dense storage bodies within the cytoplasm of neurons and many other cell types. We have recently identified sphingolipid activator proteins A and D as major components of the storage cytosomes in the infantile form of NCL. Using an immunological approach we have shown that sphingolipid activator proteins also constitute an integral component of the storage bodies in the other major forms of the disease.

• Authors: M Haltia, J Tyynelä, M Baumann, M Henseler, K Sandhoff
• Journal: Gerontology (Gerontology) Vol. 41 Suppl 2 Pg. 239-48 ( 1995) ISSN: 0304-324X [Print] Switzerland
• PMID: 8821335 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

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

Topics

• Blotting, Western
• Brain (ultrastructure)
• Culture Techniques
• Glycoproteins (analysis, metabolism)
• Humans
• Immunohistochemistry
• Microscopy, Electron
• Neuronal Ceroid-Lipofuscinoses (immunology, pathology)
• Saposins
• Sphingolipid Activator Proteins
• Sphingolipids (metabolism)