Intracellular serpins, firewalls and tissue necrosis

Trends Cell Biol. 2008 Feb;18(2):45-7. doi: 10.1016/j.tcb.2007.11.006. Epub 2008 Jan 22.

Abstract

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.

MeSH terms

  • Angioedema / enzymology
  • Emphysema / enzymology
  • Humans
  • Intracellular Fluid / enzymology*
  • Liver Diseases / enzymology*
  • Lysosomes / enzymology
  • Lysosomes / pathology
  • Models, Molecular
  • Mutation
  • Necrosis / enzymology*
  • Neurodegenerative Diseases / enzymology*
  • Protein Conformation
  • Protein Structure, Tertiary
  • Serpins* / classification
  • Serpins* / genetics
  • Serpins* / physiology
  • Thrombosis / enzymology*

Substances

  • Serpins