B lymphocytes are the effectors of humoral immunity, providing defense against pathogens through different functions including antibody production. B cells constitute approximately 15% of peripheral blood leukocytes and arise from hemopoietic stem cells in the bone marrow. It is here that their antigen receptors (surface immunoglobulin) are assembled. In the context of autoimmune diseases defined by B and/or T cell autoreactive that upon activation lead to chronic tissue inflammation and often irreversible structural and functional damage, B lymphocytes play an essential role by not only producing autoantibodies but also functioning as antigen-presenting cells (APC) and as a source of cytokines. In this paper, we describe B lymphocyte functions in autoimmunity and autoimmune diseases with a special focus on their abnormalities in systemic lupus erythematosus. 1. Introduction Systemic lupus erythematosus (SLE) is the prototype of the systemic autoimmune diseases characterized by multiorgan involvement. This systemic compromise is mediated by a global loss of self-tolerance. The loss of tolerance is a consequence of genetic factors, in the context of specific environmental triggers, with the subsequent development of an altered immune response. Both innate and acquired immune mechanisms are implicated in the disease pathogenesis. Recently, special attention has been focused on the B cell abnormalities. In this paper, we will describe the B cell development, tolerance mechanism, and their implications in autoimmune diseases, with emphasis on SLE. 2. B Cell Development and the B Cell Receptor Formation Different populations of B cells result in preimmune pools where each cell in these quiescent populations expresses a B cell antigen receptor (BCR) with a unique specificity. When the BCRs come in contact with their specific antigen, several intracellular signals are generated leading to activation, differentiation, and formation of plasma cells and memory B cells. This last subset of B cells maintains protective antibody levels and mediates the response to subsequent antigen challenges. As the mechanisms leading to maturing and antibody production are complex, the alterations of some of these populations or critical steps have been associated with immunodeficiency and autoimmune diseases. Table 1 summarizes the most important features of each of the subpopulations (lineages) of B lymphocytes [1]. Table 1: Characteristics of primary B cell subsets and their progenitors. 2.1. B Cell Development This process begins from stem cells present in the bone marrow
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