Article
Accumulation of sphingolipids in SAP-precursor (prosaposin)-deficient fibroblasts occurs as intralysosomal membrane structures and can be completely reversed by treatment with human SAP-precursor.
European journal of cell biology - 1 May 1997
Burkhardt J K, Hüttler S, Klein A, Möbius W, Habermann A, Griffiths G, Sandhoff K
Abstract excerpt
The degradation of glycosphingolipids takes place in lysosomes by action of specific exohydrolases, with the assistance of sphingolipid activator proteins (SAPs). Four of the SAPs, SAP-A to -D (also called saposins A to D), are synthesized from a single protein, the SAP-precursor (prosaposin). De...
Topics
- Endosomes
- Fibroblasts
- Glycoproteins
- Glycosphingolipids
- Humans
- Inclusion Bodies
- Infant
- Intracellular Membranes
- Lysosomal Storage Diseases
- Lysosomes
- Phenotype
- Protein Precursors
- Saposins