Defective lysosomal exocytosis and plasma membrane repair in Chediak-Higashi/beige cells.

ABSTRACT: Plasma membrane resealing is a Ca(2+)-dependent process that involves the exocytosis of intracellular vesicles next to the wound site. Recent studies revealed that conventional lysosomes behave as Ca(2+)-regulated secretory compartments and play a central role in membrane resealing. These findings raised the possibility that the complex pathology of lysosomal diseases might also include defects in plasma membrane repair. Here, we investigated the capacity for lysosomal exocytosis and membrane resealing of fibroblasts derived from Chediak-Higashi syndrome (CHS) patients, or from beige-J mice. By using a sensitive electroporation/fluorescence-activated cell sorter-based assay, we show that lysosomal exocytosis triggered by membrane wounding is impaired in both human Chediak-Higashi and mouse

SUBMITTER: Huynh C 

PROVIDER: S-EPMC534728 | biostudies-literature | 2004 Nov

REPOSITORIES: biostudies-literature