The pre-B cell receptor checkpoint

Abstract 

B lymphocytes are essential antibody-producing cells of the immune system. During the development of progenitor B cells to mature B cells that express a membrane-bound antibody, the B cell receptor (BCR), the cells undergo selection at several checkpoints, which ensures that a diverse antibody repertoire is generated and that the BCRs recognise foreign-, but not self-, antigens. In this review, we consider the pre-BCR checkpoint. Mutations or alterations that affect this checkpoint underpin the development of pre-B cell leukemias, primary immunodeficiency, and possibly, systemic autoimmunity.

Abbreviations: aa, amino acids, BCR, B cell receptor, CD, cluster of differentiation, CDR, complementarity determining region, D, diversity, H chain, heavy chain, Ig, immunoglobulin, J, joining, L chain, light chain, λ5T, λ5-deficient mice, λ5-UR, λ5 unique region, μMT, mice lacking the transmembrane region of μH chain, pre-BCR, pre-B cell receptor, RAG, recombination-activating genes, SL chain, surrogate light chain, SLE, systemic lupus erythematosus, V, variable

Keywords: Antibody, B cell, Pre-B cell receptor checkpoint, Positive selection, Negative selection