EMBO Member
Konrad Sandhoff
EMBO 2000
Universität, Bonn, Germany
Reconstitution of lysosomal membrane degradation
Glycosphingolipids and sphingolipids are amphiphilic components of cellular plasma membranes. After endocytosis, they reach intraendosomal and intralysosomal vesicles for degradation. We analyze the complex and strict regulation of their catabolism by membrane lipids and lipid binding and transfer proteins, the SAPs (sphingolipid activator proteins) to understand the molecular and cellular pathology of fatal sphingolipid storage diseases.
Keywords
Sphingolipid metabolism / lysosomal diseases / lipid transfer proteins / membrane digestion / skin permeability barrier