Absence of functional fetal regulatory T cells in humans causes in utero organ-specific autoimmunity

J Allergy Clin Immunol. 2017 Aug;140(2):616-619.e7. doi: 10.1016/j.jaci.2017.02.017. Epub 2017 Mar 16.

Abstract

Regulatory T cells play a critical role in preventing fetal organ-specific autoimmunity in humans. Autopsies of neonatal IPEX patients shortly after birth demonstrate chronic exocrine-dominant pancreatitis with tertiary lymphoid structures containing expanded oligoclonal T/B lymphocytes.

Publication types

  • Case Reports
  • Letter
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Autoimmunity*
  • Biomarkers
  • Disease Models, Animal
  • Fetal Development / immunology*
  • Humans
  • Immunohistochemistry
  • Immunologic Deficiency Syndromes / diagnosis
  • Immunologic Deficiency Syndromes / etiology
  • Immunologic Deficiency Syndromes / metabolism
  • Infant, Newborn
  • Mice
  • Mice, Transgenic
  • Organ Specificity / immunology
  • T-Lymphocytes, Regulatory / immunology*
  • T-Lymphocytes, Regulatory / metabolism

Substances

  • Biomarkers