Severe impairment of interleukin-1 and Toll-like receptor signalling in mice lacking IRAK-4

Nature. 2002 Apr 18;416(6882):750-6. doi: 10.1038/nature736. Epub 2002 Mar 31.

Abstract

Toll-like receptors (TLRs), which recognize pathogen-associated molecular patterns, and members of the pro-inflammatory interleukin-1 receptor (IL-1R) family, share homologies in their cytoplasmic domains called Toll/IL-1R/plant R gene homology (TIR) domains. Intracellular signalling mechanisms mediated by TIRs are similar, with MyD88 (refs 5-8) and TRAF6 (refs 9, 10) having critical roles. Signal transduction between MyD88 and TRAF6 is known to involve the serine-threonine kinase IL-1 receptor-associated kinase 1 (IRAK-1) and two homologous proteins, IRAK-2 (ref. 12) and IRAK-M. However, the physiological functions of the IRAK molecules remain unclear, and gene-targeting studies have shown that IRAK-1 is only partially required for IL-1R and TLR signalling. Here we show by gene-targeting that IRAK-4, an IRAK molecule closely related to the Drosophila Pelle protein, is indispensable for the responses of animals and cultured cells to IL-1 and ligands that stimulate various TLRs. IRAK-4-deficient animals are completely resistant to a lethal dose of lipopolysaccharide (LPS). In addition, animals lacking IRAK-4 are severely impaired in their responses to viral and bacterial challenges. Our results indicate that IRAK-4 has an essential role in innate immunity.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Arenaviridae Infections / immunology
  • Arenaviridae Infections / metabolism
  • B-Lymphocytes / drug effects
  • B-Lymphocytes / immunology
  • B-Lymphocytes / metabolism
  • Cells, Cultured
  • Drosophila Proteins*
  • Gene Deletion
  • Immunity, Innate / immunology
  • Interferon-gamma / analysis
  • Interleukin-1 / biosynthesis
  • Interleukin-1 / pharmacology
  • Interleukin-1 Receptor-Associated Kinases
  • Interleukin-6 / biosynthesis
  • JNK Mitogen-Activated Protein Kinases
  • Killer Cells, Natural / drug effects
  • Killer Cells, Natural / immunology
  • Ligands
  • Lipopolysaccharides / pharmacology
  • Lymphocytic choriomeningitis virus / physiology
  • Macrophages / drug effects
  • Macrophages / immunology
  • Macrophages / metabolism
  • Membrane Glycoproteins / metabolism*
  • Mice
  • Mitogen-Activated Protein Kinases / metabolism
  • NF-kappa B / metabolism
  • Nitric Oxide / metabolism
  • Protein Kinases / deficiency*
  • Protein Kinases / genetics
  • Protein Kinases / metabolism*
  • Receptors, Cell Surface / metabolism*
  • Receptors, Interleukin-1 / metabolism*
  • Signal Transduction* / drug effects
  • Staphylococcal Infections / immunology
  • Staphylococcal Infections / metabolism
  • Staphylococcus aureus / physiology
  • Toll-Like Receptors
  • Tumor Necrosis Factor-alpha / biosynthesis
  • Tumor Necrosis Factor-alpha / pharmacology
  • p38 Mitogen-Activated Protein Kinases

Substances

  • Drosophila Proteins
  • Interleukin-1
  • Interleukin-6
  • Ligands
  • Lipopolysaccharides
  • Membrane Glycoproteins
  • NF-kappa B
  • Receptors, Cell Surface
  • Receptors, Interleukin-1
  • Toll-Like Receptors
  • Tumor Necrosis Factor-alpha
  • Nitric Oxide
  • Interferon-gamma
  • Protein Kinases
  • Interleukin-1 Receptor-Associated Kinases
  • Irak3 protein, mouse
  • JNK Mitogen-Activated Protein Kinases
  • Mitogen-Activated Protein Kinases
  • p38 Mitogen-Activated Protein Kinases