Publications

Abstract

Regulated interactions between cells of the immune system facilitate the generation of successful immune responses, thereby enabling efficient neutralization and clearance of pathogens, and the establishment of both cell and humoral-mediated immunological memory. The corollary of this is that impediments to efficient cell-cell interactions compromise the differentiation and effector function of immune cells, underlying the clinical features of, and disease pathogenesis in, primary immunodeficiencies. These defects manifest as impaired long-term humoral immunity and susceptibility to infection with specific pathogens in affected individuals. In this review, we discuss the importance of, and requirements for, effective interactions between B cells and T cells during the formation of CD4+ T follicular helper cells and the elicitation of cytotoxic function of virus-specific CD8+ T cells, and how these processes are abrogated in primary immunodeficiencies due to loss-of function mutations in defined genes.