Dendritic cell defects in primary immunodeficiency disorders

Abstract

Dendritic cells (DC) are professional antigen-presenting cells that play a key role in linking the innate and adaptive arms of the immune system. In vitro, DC perform critical functions such as antigen uptake and processing, priming of naïve T cells and production of cytokines to regulate other immune cells. In vivo experimental systems support a central role for DC in inducing protective immune responses but the effect of DC deficiency in existing whole animal models is smaller than would be predicted. Studies of human primary immunodeficiency disorders (PID) have significantly advanced our understanding of the development and function of other immune cells and provide some important information about DC. Although only a small number of rare monogenic PID that cause DC deficiency have been described to date, impaired DC function forms part of the immunophenotype of several PID and is likely to contribute to clinical presentation. This review focuses on what is known so far about the role of DC in PID and what implications this has for basic DC biology.