Stress-induced phosphorylation and proteasomal degradation of mitofusin 2 facilitates mitochondrial fragmentation and apoptosis

Mol Cell. 2012 Aug 24;47(4):547-57. doi: 10.1016/j.molcel.2012.05.041. Epub 2012 Jun 28.

Abstract

Mitochondria play central roles in integrating pro- and antiapoptotic stimuli, and JNK is well known to have roles in activating apoptotic pathways. We establish a critical link between stress-induced JNK activation, mitofusin 2, which is an essential component of the mitochondrial outer membrane fusion apparatus, and the ubiquitin-proteasome system (UPS). JNK phosphorylation of mitofusin 2 in response to cellular stress leads to recruitment of the ubiquitin ligase (E3) Huwe1/Mule/ARF-BP1/HectH9/E3Histone/Lasu1 to mitofusin 2, with the BH3 domain of Huwe1 implicated in this interaction. This results in ubiquitin-mediated proteasomal degradation of mitofusin 2, leading to mitochondrial fragmentation and enhanced apoptotic cell death. The stability of a nonphosphorylatable mitofusin 2 mutant is unaffected by stress and protective against apoptosis. Conversely, a mitofusin 2 phosphomimic is more rapidly degraded without cellular stress. These findings demonstrate how proximal signaling events can influence both mitochondrial dynamics and apoptosis through phosphorylation-stimulated degradation of the mitochondrial fusion machinery.

Publication types

  • Research Support, N.I.H., Intramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Apoptosis / physiology*
  • BH3 Interacting Domain Death Agonist Protein / metabolism
  • Cell Line, Tumor
  • GTP Phosphohydrolases / metabolism*
  • Humans
  • MAP Kinase Kinase 4 / metabolism
  • Mitochondria / enzymology
  • Mitochondria / metabolism*
  • Mitochondrial Proteins / metabolism*
  • Phosphorylation
  • Proteasome Endopeptidase Complex / metabolism*
  • Proteolysis
  • Stress, Physiological / physiology*
  • Tumor Suppressor Proteins
  • Ubiquitin / metabolism
  • Ubiquitin-Protein Ligases / metabolism
  • Ubiquitination / physiology

Substances

  • BH3 Interacting Domain Death Agonist Protein
  • BID protein, human
  • Mitochondrial Proteins
  • Tumor Suppressor Proteins
  • Ubiquitin
  • HUWE1 protein, human
  • Ubiquitin-Protein Ligases
  • MAP Kinase Kinase 4
  • Proteasome Endopeptidase Complex
  • GTP Phosphohydrolases
  • MFN2 protein, human