CD20 Deficiency, Lessons Related to Therapeutic Biologicals and Primary Immunodeficiency
CD20 was one of the first B-cell-specific differentiation antigens identified (Stashenko et al. 1980). Nowadays, anti-CD20 immunotherapy using chimeric monoclonal antibodies (MoAbs) is used for the treatment of B-cell neoplasia, EBV-associated immunopathology, and a growing list of diseases with presumed autoimmune origin (Franks et al. 2016; Miano 2016; Claes et al. 2015; Froissart et al. 2015).
CD20, encoded by membrane-spanning 4 domains, subfamily a, member 1 (MS4A1; OMIM*112210), belongs to the MS4A family of molecules with multiple membrane-spanning domains (Liang et al. 2001; Liang and Tedder 2001) and is expressed on pre-B and mature B cells but is downregulated upon differentiation into plasma cells (Tedder and Engel 1994). CD20 is unlikely to have a natural ligand, but in vitro studies with CD20, MoAbs have demonstrated its involvement in the regulation of B-cell activation and proliferation (Tedder et al. 1985, 1986).
The CD20gene structure and expression...
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