Article
A mutation in X-linked inhibitor of apoptosis (G466X) leads to memory inflation of Epstein-Barr virus-specific T cells.
Clinical and experimental immunology - 1 Dec 2014
Lopez-Granados E, Stacey M, Kienzler A-K, Sierro S, Willberg C B, Fox C P, Rigaud S, Long H M, Hislop A D, Rickinson A B, Patel S, Latour S, Klenerman P, Chapel H
Abstract excerpt
Mutations in the X-linked inhibitor of apoptosis (XIAP) gene have been associated with XLP-like disease, including recurrent Epstein-Barr virus (EBV)-related haemophagocytic lymphohystiocytosis (HLH), but the immunopathogenic bases of EBV-related disease in XIAP deficiency is unknown. We present the first analysis of EBV-specific T cell responses in functional XIAP deficiency. In a family of patients with a novel...
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