Intracellular serpins, firewalls and tissue necrosis.

Research paper by Stefan J SJ Marciniak, David A DA Lomas

Indexed on: 25 Jan '08Published on: 25 Jan '08Published in: Trends in Cell Biology


Luke and colleagues have recently attributed a new role to a member of the serpin superfamily of serine proteinase inhibitors. They have used Caenorhabditis elegans to show that an intracellular serpin is crucial for maintaining lysosomal integrity. We examine the role of this firewall in preventing necrosis and attempt to integrate this with current theories of stress-induced protein degradation. We discuss how mutant serpins cause disease either through polymerization or now, perhaps, by unleashing necrosis.