STAT6 deficiency ameliorates Graves' disease severity by suppressing thyroid epithelial cell hyperplasia

Cell Death Dis. 2016 Dec 1;7(12):e2506. doi: 10.1038/cddis.2016.398.

Abstract

Signal transducer and activator of transcription 6 (STAT6) is involved in epithelial cell growth. However, little is known regarding the STAT6 phosphorylation status in Graves' disease (GD) and its role in thyroid epithelial cells (TECs). In this study, we found that STAT6 phosphorylation (p-STAT6) was significantly increased in TECs from both GD patients and experimental autoimmune Graves' disease mice and that STAT6 deficiency ameliorated GD symptoms. Autocrine IL-4 signalling in TECs activated the phosphorylation of STAT6 via IL-4 R engagement, and the downstream targets of STAT6 were Bcl-xL and cyclin D1. Thus, the IL-4-STAT6-Bcl-xL/cyclin D1 pathway is crucial for TEC hyperplasia, which aggravates GD. More importantly, in vitro and in vivo experiments demonstrated that STAT6 phosphorylation inhibited by AS1517499 decreased TEC hyperplasia, thereby reducing serum T3 and T4 and ameliorating GD. Thus, our study reveals that in addition to the traditional pathogenesis of GD, in which autoantibody TRAb stimulates thyroid-stimulating hormone receptors and consequently produces T3, T4, TRAb could also trigger TECs producing IL-4, and IL-4 then acts in an autocrine manner to activate p-STAT6 signalling and stimulate unrestricted cell growth, thus aggravating GD. These findings suggest that STAT6 inhibitors could be potent therapeutics for treating GD.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cyclin D1 / metabolism
  • Graves Disease / metabolism*
  • Graves Disease / pathology*
  • Humans
  • Hyperplasia
  • Interleukin-4 / metabolism
  • Mice, Inbred BALB C
  • Models, Biological
  • Phosphorylation / drug effects
  • Pyrimidines / pharmacology
  • Receptors, Interleukin / metabolism
  • STAT6 Transcription Factor / deficiency*
  • STAT6 Transcription Factor / metabolism
  • Severity of Illness Index*
  • Thyroid Epithelial Cells / drug effects
  • Thyroid Epithelial Cells / metabolism*
  • Thyroid Epithelial Cells / pathology*
  • Up-Regulation / drug effects
  • bcl-X Protein / metabolism

Substances

  • 4-(benzylamino)-2-((2-(3-chloro-4-hydroxyphenyl)ethyl)amino)pyrimidine-5-carboxamide
  • Pyrimidines
  • Receptors, Interleukin
  • STAT6 Transcription Factor
  • bcl-X Protein
  • Cyclin D1
  • Interleukin-4