Epidermal loss of JunB leads to a SLE phenotype due to hyper IL-6 signaling

Proc Natl Acad Sci U S A. 2009 Dec 1;106(48):20423-8. doi: 10.1073/pnas.0910371106. Epub 2009 Nov 16.

Abstract

Systemic lupus erythematosus (SLE) is a complex autoimmune disease affecting various tissues. Involvement of B and T cells as well as increased cytokine levels have been associated with disease manifestation. Recently, we demonstrated that mice with epidermal loss of JunB (JunB(Deltaep)) develop a myeloproliferative syndrome (MPS) due to high levels of G-CSF which are secreted by JunB-deficient keratinocytes. In addition, we show that JunB(Deltaep) mice develop a SLE phenotype linked to increased epidermal interleukin 6 (IL-6) secretion. Intercrosses with IL-6-deficient mice could rescue the SLE phenotype. Furthermore, we show that JunB binds to the IL-6 promoter and transcriptionally suppresses IL-6. Facial skin biopsies of human SLE patients similarly revealed low JunB protein expression and high IL-6, activated Stat3, Socs-1, and Socs-3 levels within lupus lesions. Thus, keratinocyte-induced IL-6 secretion can cause SLE and systemic autoimmunity. Our results support trials to use alpha-IL-6 receptor antibody therapy for treatment of SLE.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Chromatin Immunoprecipitation
  • Crosses, Genetic
  • Enzyme-Linked Immunosorbent Assay
  • Epidermis / metabolism*
  • Fluorescent Antibody Technique, Direct
  • Gene Expression Regulation / physiology*
  • Granulocyte Colony-Stimulating Factor / metabolism
  • Humans
  • Interleukin-6 / metabolism
  • Keratinocytes / metabolism*
  • Luciferases
  • Lupus Erythematosus, Systemic / metabolism
  • Lupus Erythematosus, Systemic / pathology*
  • Mice
  • Mice, Transgenic
  • Microscopy, Electron
  • Proto-Oncogene Proteins c-jun / deficiency*
  • Proto-Oncogene Proteins c-jun / metabolism

Substances

  • Interleukin-6
  • Proto-Oncogene Proteins c-jun
  • Granulocyte Colony-Stimulating Factor
  • Luciferases