The mitochondrial protease HtrA2 is regulated by Parkinson's disease-associated kinase PINK1

Nat Cell Biol. 2007 Nov;9(11):1243-52. doi: 10.1038/ncb1644. Epub 2007 Sep 30.

Abstract

In mice, targeted deletion of the serine protease HtrA2 (also known as Omi) causes mitochondrial dysfunction leading to a neurodegenerative disorder with parkinsonian features. In humans, point mutations in HtrA2 are a susceptibility factor for Parkinson's disease (PARK13 locus). Mutations in PINK1, a putative mitochondrial protein kinase, are associated with the PARK6 autosomal recessive locus for susceptibility to early-onset Parkinson's disease. Here we determine that HtrA2 interacts with PINK1 and that both are components of the same stress-sensing pathway. HtrA2 is phosphorylated on activation of the p38 pathway, occurring in a PINK1-dependent manner at a residue adjacent to a position found mutated in patients with Parkinson's disease. HtrA2 phosphorylation is decreased in brains of patients with Parkinson's disease carrying mutations in PINK1. We suggest that PINK1-dependent phosphorylation of HtrA2 might modulate its proteolytic activity, thereby contributing to an increased resistance of cells to mitochondrial stress.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Binding Sites / genetics
  • Brain / pathology
  • Cell Line
  • Enzyme Activation / genetics
  • High-Temperature Requirement A Serine Peptidase 2
  • Humans
  • MAP Kinase Kinase Kinase 3 / metabolism
  • Mice
  • Mitochondrial Proteins / genetics
  • Mitochondrial Proteins / physiology*
  • Models, Biological
  • Mutagenesis, Site-Directed
  • Mutation
  • Parkinson Disease / etiology*
  • Phosphorylation
  • Protein Kinases / genetics
  • Protein Kinases / physiology*
  • Serine Endopeptidases / genetics
  • Serine Endopeptidases / physiology*
  • Signal Transduction

Substances

  • Mitochondrial Proteins
  • Protein Kinases
  • PTEN-induced putative kinase
  • MAP Kinase Kinase Kinase 3
  • MAP3K3 protein, human
  • Serine Endopeptidases
  • HTRA2 protein, human
  • High-Temperature Requirement A Serine Peptidase 2
  • Htra2 protein, mouse