Targeting RIPK3 oligomerization blocks necroptosis without inducing apoptosis

FEBS Lett. 2020 Jul;594(14):2294-2302. doi: 10.1002/1873-3468.13812. Epub 2020 Jun 1.

Abstract

Receptor-interacting serine/threonine-protein kinase 3 (RIPK3) is a central protein in necroptosis with great potential as a target for treating necroptosis-associated diseases, such as Crohn's disease. However, blockade of RIPK3 kinase activity leads to unexpected RIPK3-initiated apoptosis. Herein, we found that PP2, a known SRC inhibitor, inhibits TNF-α-induced necroptosis without initiating apoptosis. Further investigation showed that PP2 acts as an inhibitor of not only SRC but also RIPK3. PP2 does not disturb the integrity of the RIPK1-RIPK3-mixed lineage kinase domain-like pseudokinase (MLKL) necroptosome or the autophosphorylation of RIPK3 at T231/S232 but disrupts RIPK3 oligomerization, thereby impairing the phosphorylation and oligomerization of MLKL. These results demonstrate the essential role of RIPK3 oligomerization in necroptosis and suggest a potential RIPK3 oligomerization-targeting strategy for therapeutic development.

Keywords: RIPK3; apoptosis; necroptosis; oligomerization; phosphorylation.

Publication types

  • Letter
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Apoptosis* / drug effects
  • Cell Line, Tumor
  • Mice
  • Necroptosis / drug effects*
  • Phosphorylation / drug effects
  • Protein Multimerization / drug effects*
  • Pyrimidines / pharmacology*
  • Receptor-Interacting Protein Serine-Threonine Kinases / chemistry*
  • Receptor-Interacting Protein Serine-Threonine Kinases / metabolism*
  • Tumor Necrosis Factor-alpha / pharmacology
  • src-Family Kinases / antagonists & inhibitors

Substances

  • AG 1879
  • Pyrimidines
  • Tumor Necrosis Factor-alpha
  • src-Family Kinases
  • Receptor-Interacting Protein Serine-Threonine Kinases
  • Ripk3 protein, mouse