Advertisement
Research Article Free access | 10.1172/JCI113371
Department of Pediatrics, University of California, Los Angeles School of Medicine 90024.
Find articles by Clement, L. in: JCI | PubMed | Google Scholar
Department of Pediatrics, University of California, Los Angeles School of Medicine 90024.
Find articles by Plaeger-Marshall, S. in: JCI | PubMed | Google Scholar
Department of Pediatrics, University of California, Los Angeles School of Medicine 90024.
Find articles by Haas, A. in: JCI | PubMed | Google Scholar
Department of Pediatrics, University of California, Los Angeles School of Medicine 90024.
Find articles by Saxon, A. in: JCI | PubMed | Google Scholar
Department of Pediatrics, University of California, Los Angeles School of Medicine 90024.
Find articles by Martin, A. in: JCI | PubMed | Google Scholar
Published March 1, 1988 - More info
The bare lymphocyte syndrome is a rare combined immunodeficiency disorder associated with the absence of class I and/or class II major histocompatibility (MHC) antigens. Although it has been inferred that the immune deficiency is a consequence of disordered MHC-restricted interactions among otherwise normal cells, the biological capabilities and differentiation of B lymphocytes deficient in class II MHC antigens have not been rigorously analyzed. We have examined the phenotypic and functional attributes of B cells with absent class II MHC antigens. Our data demonstrate that these B cells are intrinsically defective in their responses to membrane-mediated activation stimuli. In addition, virtually all the B cells had phenotypic evidence of arrested differentiation at an immature stage. Finally, these B cells also failed to express the C3d-EBV receptor normally present on all B lymphocytes. These data indicate that class II MHC molecules are vital participants in early events of the B cell activation cascade, and that other non-MHC membrane molecules may also be absent as a consequence of either arrested differentiation or as a result of the basic defect affecting the expression of MHC membrane antigens.