Exaggerated follicular helper T-cell responses in patients with LRBA deficiency caused by failure of CTLA4-mediated regulation


Alroqi F. J. , Charbonnier L., BARIŞ S., KIYKIM A., Chou J., Platt C. D. , ...More

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, vol.141, no.3, pp.1050-1069, 2018 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 141 Issue: 3
  • Publication Date: 2018
  • Doi Number: 10.1016/j.jaci.2017.05.022
  • Journal Name: JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.1050-1069
  • Keywords: Autoantibodies, LPS-responsive beige-like anchor, cytotoxic T lymphocyte-associated antigen 4, regulatory T cells, follicular helper T cells, follicular regulatory T cells, SYSTEMIC-LUPUS-ERYTHEMATOSUS, IMMUNE DYSREGULATION, MUTATIONS, AUTOIMMUNITY, CTLA-4, DIFFERENTIATION, DISEASE, PHENOTYPE, EXPANSION, PROMOTE
  • Istanbul University-Cerrahpasa Affiliated: Yes

Abstract

Background: LPS-responsive beige-like anchor protein (LRBA) and cytotoxic T lymphocyte-associated antigen 4 (CTLA4) deficiencies give rise to overlapping phenotypes of immune dysregulation and autoimmunity, with dramatically increased frequencies of circulating follicular helper T (cTFH) cells.