Published January 1, 2018 | Version v1
Journal article Open

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

  • 1. Marmara Univ, Div Pediat Allergy Immunol, Istanbul, Turkey
  • 2. King Faisal Specialist Hosp & Res Ctr, Dept Pediat, Riyadh, Saudi Arabia
  • 3. King Faisal Specialist Hosp & Res Ctr, Dept Genet, Riyadh, Saudi Arabia

Description

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.

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