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Review

Immune Checkpoint Dysfunction Remodels Adaptive Immune Receptor Repertoires in CTLA-4 Insufficiency and LRBA Deficiency

Gökhan Cildir Selin Cildir Rahmi Kutay Erdogan Umran Aba Şule Haskoloğlu S. Bozkurt N. Ozturk C. Aytekin Nicholas I. Warnock C. Kok Damla Pehlivan Canberk Ipsir Ceren Yarimkaya B. Karataş Nihan Doga Kozanli Feyza Bayram Catak Mehmet Cihangir Çatak G. Kılınç G. Esendagli Hamdullah Yanık D. Ozcan M. Serbes Candan Islamoğlu S. S. Kilic Yasin Karalı S. Can M. Y. Altunbas E. Karabıber Çiğdem Aydoğmuş Sait Karaman F. Çelmeli G. Yildiz Emine Vezir Gulay Irem Parlak Özel S. Keleş I. Reisli Ş. Guner M. Özbey A. Yıldıran S. Karadag N. Gulez F. Genel F. Çelik Özgen Soyöz N. Karaca G. Aksu T. Arıkoğlu A. Ozhan S. Kuyucu Sevgi Bilgic Eltan E. Karakoc-Aydiner I. Tezcan F. Doğu A. Ikincioğullari A. Ozen S. Barış B. Erman
Oct 2026 · Clinical reviews in allergy and immunology · Vol 69 · 0 citations · 75 references
Medicine

TL;DR

It is demonstrated that inherited disruption of the CTLA-4/LRBA axis profoundly remodels AIRRs, preferentially affecting CD8⁺ T-cell diversity while also constraining humoral immunity, and highlight a central role for this immune checkpoint pathway in maintaining adaptive immune repertoire diversity and clonal homeostasis.

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