BAFF Receptor Deficiency

SMULSKI, C. R.; ODERMATT, P.; EIBEL, H.

Humoral Primary Immunodeficiencies

Springer

Año: 2018; p. 137 - 153

Starting from hematopoietic stem cells, B lymphocytes develop in human bonemarrow through successive developmental stages. Progression through these stagesis mainly regulated by the rearrangement of the variable parts of the immunoglobulinheavy (H) and light (L) chains. After the successful rearrangement of H- andL-chain loci, functional IgM molecules are assembled and expressed together withthe signal-transducing components Ig-α and Ig-β in the form of B cell antigen receptorson the cell surface of immature IgM+ B cells. Since the rearrangement of immunoglobulingene segments allows the generation of autoreactive receptors, theself-reactivity of IgM molecules expressed is tested and eventually corrected byreceptor editing in immature B cells. Then, only those cells with low avidity to self-antigenscan leave the bone marrow and migrate as so-called transitional B cells tothe spleen where they conclude their early, antigen-independent steps of B celldevelopment (reviewed in [1]).