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
Highlights What are the main findings? This tertiary-center cohort supports viewing pediatric DOCK8 deficiency as an actin cytoskeleton-related IEI with overlapping infectious, allergic, immune-dysregulatory, and systemic manifestations. Beyond eczema and recurrent infections, the cohort included cytopenias, autoimmuni...
Figen Çelebi Çelik, N. Yüksel, Ömer Akçal et al.· Children· 0 citations
Inborn errors of immunity (IEIs) are a heterogeneous group of genetic disorders in which timely diagnosis is essential for appropriate treatment and improved clinical outcomes. Although next-generation sequencing provides definitive molecular confirmation, it is often time-consuming, costly, and may not identify al...
Özgen Soyöz, Figen Çelebi Çelik, M. Kaya et al.· Frontiers in Immunology· 0 citations
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