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

Alroqi, Fayhan; Charbonnier, Louis-Marie; BARIŞ, SAFA; Kiykim, Ayca; Chou, Janet; Platt, Craig; Algassim, Abdulrahman; Keles, Sevgi; Al Saud, Bandar; Alkuraya, Fowzan; Jordan, Michael; Geha, Raif; Chatila, Talal

doi:10.1016/j.jaci.2017.05.022

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., ...Daha Fazla

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, cilt.141, sa.3, ss.1050-1069, 2018 (SCI-Expanded)

Yayın Türü: Makale / Tam Makale
Cilt numarası: 141 Sayı: 3
Basım Tarihi: 2018
Doi Numarası: 10.1016/j.jaci.2017.05.022
Dergi Adı: JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
Sayfa Sayıları: ss.1050-1069
Anahtar Kelimeler: 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
Marmara Üniversitesi Adresli: Evet

Özet

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.