Article
HyperIgE in hypomorphic recombination-activating gene defects.
Current opinion in immunology - 1 Feb 2023
Castiello Maria Carmina, Brandas Chiara, Capo Valentina, Villa Anna
Abstract excerpt
Increased immunogloblulin-E (IgE) levels associated with eosinophilia represent a common finding observed in Omenn syndrome, a severe immunodeficiency caused by decreased V(D)J recombination, leading to restricted T- and B-cell receptor repertoire. V(D)J recombination is initiated by the lymphoid-restricted recombination-activating gene (RAG) recombinases. The lack of RAG proteins causes a block in lymphocyte...
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