T cell help to B cells is a fundamental aspect of adaptive immunity and the generation of
immunological memory. Follicular helper CD4 T (TFH) cells are the specialized providers of …

The hyper IgM syndromes are a group of rare inherited immune deficiency disorders
characterized by impairment of immunoglobulin isotype switching resulting from defects in …

Background Crohn's disease is associated with excess cytokine activity mediated by type 1
helper T (Th1) cells. Interleukin-12 is a key cytokine that initiates Th1-mediated inflammatory …

The X-linked hyper-IgM (XHIGM) syndrome is an uncommon primary immunodeficiency
disease caused by mutations in the gene for CD40 ligand and characterized by normal or …

The gene that encodes nuclear factor κ (NF-κB) essential modulator (or NEMO, also known
as IKKγ) is required for activation of the transcription factor NF-κB. We describe mutations in …

Background X-linked hyper-IgM syndrome (XHIGM) is a primary immunodeficiency with high
morbidity and mortality compared with those seen in healthy subjects. Hematopoietic cell …

Healthy young children who acquire CMV have prolonged viral shedding into the urine and
saliva, but whether this is attributable to limitations in viral-specific immune responses has …

Precise correction of the CD40LG gene in T cells and hematopoietic stem/progenitor cells
(HSPC) holds promise for treating X‐linked hyper‐IgM Syndrome (HIGM1), but its actual …

The CD28-B7 and CD154-CD40 pathways have been described as the critical costimulatory
pathways for T cell activation. Blockade of these pathways has been reported to regulate …

Abstract Purpose The United States Immunodeficiency Network (USIDNET) patient registry
was used to characterize the presentation, genetics, phenotypes, and treatment of patients …