KLHDC10 Deficiency Protects Mice against TNFα-Induced Systemic Inflammation

PLoS One. 2016 Sep 15;11(9):e0163118. doi: 10.1371/journal.pone.0163118. eCollection 2016.

Abstract

Systemic inflammatory response syndrome (SIRS) is a form of fatal acute inflammation for which there is no effective treatment. Here, we revealed that the ablation of Kelch domain containing 10 (KLHDC10), which we had originally identified as an activator of Apoptosis Signal-regulating Kinase 1 (ASK1), protects mice against TNFα-induced SIRS. The disease development of SIRS is mainly divided into two stages. The early stage is characterized by TNFα-induced systemic necroptosis, a regulated form of necrosis mediated by Receptor-interacting protein (RIP) 1/3 kinases. The later stage presents with an over-production of inflammatory cytokines induced by damage-associated molecular patterns (DAMPs), which are immunogenic cellular contents released from cells that underwent necroptosis. Analysis of TNFα-challenged mice revealed that KLHDC10-deficient mice show a reduction in the inflammatory response, but not in early systemic necroptosis. In vitro analysis suggested that the reduced inflammatory response observed in KLHDC10-deficient mice might be caused, in part, by enhanced necroptosis of inflammatory cells encountering DAMPs. Interestingly, the enhancement of necroptosis induced by KLHDC10 deficiency was selectively observed in inflammatory cells. Our results suggest that KLHDC10 is a cell-type specific regulator of necroptosis that ultimately contributes to the development of TNFα-induced SIRS.

MeSH terms

  • Animals
  • Carrier Proteins / genetics*
  • Cell Line
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Systemic Inflammatory Response Syndrome / physiopathology*
  • Tumor Necrosis Factor-alpha / physiology*

Substances

  • Carrier Proteins
  • KLHDC10 protein, mouse
  • Tumor Necrosis Factor-alpha

Grants and funding

This work was supported by the Grant-in-Aid for Scientific Research (KAKENHI) from the Japan Society for the Promotion of Science (JSPS) and the Japanese Ministry of Education, Culture, Sports, Science and Technology (MEXT) (Grant number; 25221302 to H. Ichijo). URL; https://www.jsps.go.jp/j-grantsinaid/. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.