The recombination-activating gene 1 (RAG1) and RAG2 proteins initiate the V (D) J
recombination process, which ultimately enables the generation of T cells and B cells with a …

Mutations of the recombinase activating genes (RAG) in humans underlie a broad spectrum
of clinical and immunological phenotypes that reflect different degrees of impairment of T …

Thymic epithelial cells (TECs) help orchestrate thymopoiesis, and TEC differentiation relies
on bidirectional interactions with thymocytes. Although the molecular mediators that …

The RAG1 and RAG2 proteins initiate the process of V (D) J recombination and therefore
play an essential role in adaptive immunity. While null mutations in the RAG genes cause …

Primary immunodeficiency diseases (PID s) consist of a genetically heterogeneous group of
immune disorders that affect distinct elements of the immune system. PID patients are more …

Primary immunodeficiencies (PIDs) are inherited disorders of the immune system,
associated with a considerable increase in susceptibility to infections. PIDs can also …

Primary immunodeficiencies (PID) comprise a diverse group of clinical disorders with varied
genetic defects. Paradoxically, a substantial proportion of PID patients develop autoimmune …

Omenn syndrome (OS) is caused by hypomorphic Rag mutations and characterized by a
profound immunodeficiency associated with autoimmune-like manifestations. Both in …

Background Recombination-activating gene 1 (RAG1) deficiency results in severe combined
immunodeficiency (SCID) caused by a complete lack of T and B lymphocytes. If untreated …

Primary immunodeficiency diseases (PIDs) consist of a large group of genetic disorders that
affect distinct components of the immune system. PID patients are susceptible to infection …