The pre-B cell receptor checkpoint

• Published in: FEBS letters Vol. 584; no. 12; pp. 2572 - 2579
• Main Authors: Mårtensson, Inga-Lill, Almqvist, Nina, Grimsholm, Ola, Bernardi, Angelina I.
• Format: Journal Article
• Language: English
• Published: England Elsevier B.V 18.06.2010
• Subjects: amino acids; Animals; Antibody; Autoimmunity; Autoimmunity - genetics; B cell; B cell receptor; B-Lymphocyte; B-Lymphoid; BCR; CDR; Cell Differentiation; Cell Differentiation - immunology; Cell Membrane; Cell Membrane - immunology; cluster of differentiation; complementarity determining region; cytology; diversity; Gene Rearrangement; Gene Rearrangement, B-Lymphocyte; genetics; H chain; heavy chain; Humans; immunoglobulin; Immunologi inom det medicinska området; Immunologic Deficiency Syndromes; Immunologic Deficiency Syndromes - genetics; Immunologic Deficiency Syndromes - immunology; Immunological; immunology; Immunology in the Medical Area; joining; L chain; light chain; Mice; mice lacking the transmembrane region of μH chain; Models, Immunological; Mutation; Negative selection; Positive selection; pre-B cell receptor; Pre-B cell receptor checkpoint; Pre-B Cell Receptors; Pre-B Cell Receptors - immunology; pre-BCR; Precursor Cells; Precursor Cells, B-Lymphoid - cytology; Precursor Cells, B-Lymphoid - immunology; RAG; recombination-activating genes; Signal Transduction; Signal Transduction - immunology; SL chain; SLE; surrogate light chain; systemic lupus erythematosus; variable; λ5 T; λ5 unique region; λ5-deficient mice; λ5-UR; μMT; aa; pre-BCR; SL chain; CD; D; Antibody; Positive selection; Pre-B cell receptor checkpoint; J; Negative selection; μMT; SLE; BCR; CDR; B cell; L chain; H chain; V; RAG; λ5-UR; λ5 T; Ig
• ISSN: 0014-5793; 1873-3468; 1873-3468
• DOI: 10.1016/j.febslet.2010.04.057

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.