SHARPIN controls regulatory T cells by negatively modulating the T cell antigen receptor complex

Nat Immunol. 2016 Mar;17(3):286-96. doi: 10.1038/ni.3352. Epub 2016 Feb 1.

Abstract

SHARPIN forms a linear-ubiquitin-chain-assembly complex that promotes signaling via the transcription factor NF-κB. SHARPIN deficiency leads to progressive multi-organ inflammation and immune system malfunction, but how SHARPIN regulates T cell responses is unclear. Here we found that SHARPIN deficiency resulted in a substantial reduction in the number of and defective function of regulatory T cells (Treg cells). Transfer of SHARPIN-sufficient Treg cells into SHARPIN-deficient mice considerably alleviated their systemic inflammation. SHARPIN-deficient T cells displayed enhanced proximal signaling via the T cell antigen receptor (TCR) without an effect on the activation of NF-κB. SHARPIN conjugated with Lys63 (K63)-linked ubiquitin chains, which led to inhibition of the association of TCRζ with the signaling kinase Zap70; this affected the generation of Treg cells. Our study therefore identifies a role for SHARPIN in TCR signaling whereby it maintains immunological homeostasis and tolerance by regulating Treg cells.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Carrier Proteins / genetics
  • Carrier Proteins / immunology*
  • Colitis / immunology
  • Cytokines / immunology
  • Female
  • Flow Cytometry
  • Humans
  • Immune Tolerance / immunology
  • Immunoblotting
  • Immunoprecipitation
  • In Vitro Techniques
  • Inflammation
  • Intracellular Signaling Peptides and Proteins
  • Jurkat Cells
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Mice, Transgenic
  • NF-kappa B / immunology
  • Receptors, Antigen, T-Cell / immunology*
  • Signal Transduction
  • T-Lymphocytes, Regulatory / immunology*
  • Ubiquitination
  • ZAP-70 Protein-Tyrosine Kinase / immunology

Substances

  • Carrier Proteins
  • Cytokines
  • Intracellular Signaling Peptides and Proteins
  • NF-kappa B
  • Receptors, Antigen, T-Cell
  • Sipl1 protein, mouse
  • antigen T cell receptor, zeta chain
  • ZAP-70 Protein-Tyrosine Kinase
  • Zap70 protein, mouse