HtrA2/Omi, a sheep in wolf's clothing

Cell. 2003 Oct 31;115(3):251-3. doi: 10.1016/s0092-8674(03)00851-1.

Abstract

Mammalian mitochondrial HtrA2/Omi was originally described as an apoptosis inducer, but rather than having extra cells, mice with mutant HtrA2/Omi suffer from a neurodegenerative disease due to progressive mitochondrial damage. This suggests that instead of promoting cell death by antagonizing inhibitor of apoptosis (IAP) proteins, the primary function of HtrA2/Omi is to handle misfolded proteins in the mitochondria.

MeSH terms

  • Animals
  • Apoptosis
  • High-Temperature Requirement A Serine Peptidase 2
  • Inhibitor of Apoptosis Proteins
  • Mice
  • Mitochondria / enzymology
  • Mitochondria / physiology*
  • Mitochondrial Proteins / chemistry
  • Mitochondrial Proteins / metabolism
  • Neurodegenerative Diseases / enzymology*
  • Neurodegenerative Diseases / genetics
  • Neurodegenerative Diseases / pathology*
  • Protein Binding
  • Protein Denaturation
  • Protein Folding
  • Proteins / metabolism
  • Serine Endopeptidases / metabolism*

Substances

  • Inhibitor of Apoptosis Proteins
  • Mitochondrial Proteins
  • Proteins
  • Serine Endopeptidases
  • High-Temperature Requirement A Serine Peptidase 2
  • Htra2 protein, mouse