STAT3 is a critical cell-intrinsic regulator of human unconventional T cell numbers and function

نویسندگان

  • Robert P. Wilson
  • Megan L. Ives
  • Geetha Rao
  • Anthony Lau
  • Kathryn Payne
  • Masao Kobayashi
  • Peter D. Arkwright
  • Jane Peake
  • Melanie Wong
  • Stephen Adelstein
  • Joanne M. Smart
  • Martyn A. French
  • David A. Fulcher
  • Capucine Picard
  • Jacinta Bustamante
  • Stephanie Boisson-Dupuis
  • Paul Gray
  • Polina Stepensky
  • Klaus Warnatz
  • Alexandra F. Freeman
  • Jamie Rossjohn
  • James McCluskey
  • Steven M. Holland
  • Jean-Laurent Casanova
  • Gulbu Uzel
  • Cindy S. Ma
  • Stuart G. Tangye
  • Elissa K. Deenick
چکیده

Unconventional T cells such as γδ T cells, natural killer T cells (NKT cells) and mucosal-associated invariant T cells (MAIT cells) are a major component of the immune system; however, the cytokine signaling pathways that control their development and function in humans are unknown. Primary immunodeficiencies caused by single gene mutations provide a unique opportunity to investigate the role of specific molecules in regulating human lymphocyte development and function. We found that individuals with loss-of-function mutations in STAT3 had reduced numbers of peripheral blood MAIT and NKT but not γδ T cells. Analysis of STAT3 mosaic individuals revealed that this effect was cell intrinsic. Surprisingly, the residual STAT3-deficient MAIT cells expressed normal levels of the transcription factor RORγt. Despite this, they displayed a deficiency in secretion of IL-17A and IL-17F, but were able to secrete normal levels of cytokines such as IFNγ and TNF. The deficiency in MAIT and NKT cells in STAT3-deficient patients was mirrored by loss-of-function mutations in IL12RB1 and IL21R, respectively. Thus, these results reveal for the first time the essential role of STAT3 signaling downstream of IL-23R and IL-21R in controlling human MAIT and NKT cell numbers.

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عنوان ژورنال:

دوره 212  شماره 

صفحات  -

تاریخ انتشار 2015