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 homeosta...
Gökhan Cildir, Selin Cildir, Rahmi Kutay Erdogan et al.· Clinical reviews in allergy...· 0 citations
Signal transducer and activator of transcription 1 (STAT1) gain‐of‐function (GOF) disease is associated with chronic mucocutaneous candidiasis, infections, autoimmunity, and immune dysregulation; however, mutation‐level determinants of phenotypic variability remain incompletely defined. We aimed to describe the clinica...
B. C. Duran, C. Aytekin, M. Tsumura et al.· Scandinavian Journal of Immu...· 0 citations
Early immunologic evaluation and timely, individualized consideration of HSCT may support optimized management in children and adolescents, in whom cancer may be the first manifestation of IEI.
Nazli Deveci Demirbas, Z. Haskoloğlu, Tutku Baylan et al.· Frontiers in Immunology· 0 citations
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