A NIK-SIX signalling axis controls inflammation by targeted silencing of non-canonical NF-κB

Nature. 2019 Apr;568(7751):249-253. doi: 10.1038/s41586-019-1041-6. Epub 2019 Mar 20.

Abstract

The non-canonical NF-κB signalling cascade is essential for lymphoid organogenesis, B cell maturation, osteoclast differentiation, and inflammation in mammals1,2; dysfunction of this system is associated with human diseases, including immunological disorders and cancer3-6. Although expression of NF-κB-inducing kinase (NIK, also known as MAP3K14) is the rate-limiting step in non-canonical NF-κB pathway activation2,7, the mechanisms by which transcriptional responses are regulated remain largely unknown. Here we show that the sine oculis homeobox (SIX) homologue family transcription factors SIX1 and SIX2 are integral components of the non-canonical NF-κB signalling cascade. The developmentally silenced SIX proteins are reactivated in differentiated macrophages by NIK-mediated suppression of the ubiquitin proteasome pathway. Consequently, SIX1 and SIX2 target a subset of inflammatory gene promoters and directly inhibit the trans-activation function of the transcription factors RELA and RELB in a negative feedback circuit. In support of a physiologically pivotal role for SIX proteins in host immunity, a human SIX1 transgene suppressed inflammation and promoted the recovery of mice from endotoxic shock. In addition, SIX1 and SIX2 protected RAS/P53-driven non-small-cell lung carcinomas from inflammatory cell death induced by SMAC-mimetic chemotherapeutic agents (small-molecule activators of the non-canonical NF-κB pathway). Our findings identify a NIK-SIX signalling axis that fine-tunes inflammatory gene expression programs under both physiological and pathological conditions.

Publication types

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

MeSH terms

  • Animals
  • Antineoplastic Agents / pharmacology
  • Carcinoma, Non-Small-Cell Lung / genetics
  • Carcinoma, Non-Small-Cell Lung / metabolism
  • Carcinoma, Non-Small-Cell Lung / pathology
  • Cell Line, Tumor
  • Female
  • Fibroblasts
  • Gene Expression Regulation / drug effects
  • HEK293 Cells
  • Homeodomain Proteins / immunology
  • Homeodomain Proteins / metabolism*
  • Humans
  • Inflammation / genetics
  • Inflammation / metabolism*
  • Listeria monocytogenes / immunology
  • Male
  • Mice
  • NF-kappa B / deficiency*
  • NF-kappa B / genetics
  • NF-kappa B / metabolism*
  • NF-kappaB-Inducing Kinase
  • Nerve Tissue Proteins / immunology
  • Nerve Tissue Proteins / metabolism*
  • Promoter Regions, Genetic
  • Protein Serine-Threonine Kinases / metabolism*
  • Shigella flexneri / immunology
  • Signal Transduction* / drug effects
  • Signal Transduction* / genetics
  • Transcription Factor RelA / metabolism
  • Transcription Factor RelB / metabolism

Substances

  • Antineoplastic Agents
  • Homeodomain Proteins
  • NF-kappa B
  • Nerve Tissue Proteins
  • RELA protein, human
  • RELB protein, human
  • SIX1 protein, human
  • SIX2 protein, human
  • Transcription Factor RelA
  • Transcription Factor RelB
  • Protein Serine-Threonine Kinases