Mechanisms of Dysregulated Antibody Responses in Inborn Errors of Immunity

Inborn errors of immunity (IEI) are a heterogeneous group of immune disorders characterized by a wide spectrum of clinical manifestations, ranging from increased susceptibility to infections to autoinflammation and autoimmune diseases. Over 400 types of IEI have been identified and many of them are associated with dysregulated antibody production. Some patients with IEI have severely reduced serum levels of one or more classes of immunoglobulins (Igs) while others may show normal Ig levels but nonetheless fail to produce specific antibodies against pathogens. Patients with IEI can also produce elevated levels of antibodies including autoantibodies. Due to the complex immune environment in vivo and the heterogeneous phenotypes even within the same type of IEI, it is difficult to judge whether the defective or excessive antibody production is caused by B cell-intrinsic defects or by abnormalities in T cells or other immune cells that affect B cell activation, differentiation, class switching, etc. Elucidating the mechanisms of the dysregulated antibody response should benefit the diagnosis and treatment of IEI and aid in the identification of causative genes.
This research topic aims to explore the cellular and molecular mechanisms of dysregulated antibody responses in different types of IEI and to identify potential therapeutic targets based on these studies.
Original Research and Review articles addressing the phenotypic and functional abnormalities in B cells, T cells, and other cells that are involved in the humoral immune responses are welcome. These include, but are not limited to, the topics listed below:
1) Early B cell development, including Ig gene rearrangement and BCR repertoire
2) B cell maturation, survival, activation, Ig gene class switching, and somatic hypermutation
3) Memory B and plasma cell differentiation
4) Identification of B cell subsets associated with dysregulated antibody production
5) T cell development, activation, and differentiation into effector cells involved in antibody responses
6) Distribution and function of T cell subsets involved in antibody responses
7) Other cells and cytokines