Abstract
Two 9 year old unrelated children with recurrent bacterial infections were described previously to have a defect in polymorphonuclear leukocyte (PMN) adherence and chemotaxis, and absence of a cell associated glycoprotein (J Pediatr 101:932, 1982). A murine monoclonal antibody (Mab) designated 60.3 which recognizes a multimeric cell surface protein complex with polypeptide chains of 95, 130 and 150 kd on normal PMN, monocytes and lymphocytes did not react with the patients' cells. The addition of Mab 60.3 to normal cells produced functional defects comparable to those of our patients. In both instances lymphocytes showed depressed in vitro proliferative responses to mitogens and allogeneic cells and decreased natural killer cell activity. PMN function studies revealed loss of ability to adhere to endothelial cells in the presence of phorbol myristate acetate and depressed chemotaxis, phagocytosis and zymosan induced iodination. To further characterize the missing protein complex, we determined its relationship to cell-surface complement receptors. Both patients lacked type 3 complement receptors reactive with C3bi (CR3) but had intact C3b receptors (CR1). Similarly, incubation of normal PMN with Mab 60.3 selectively inhibited CR3-mediated rosettes. These studies indicate that the missing membrane-associated proteins recognized by Mab 60.3, among which is one with CR3 receptor activity, plays a significant role in PMN and lymphocyte function and that its absence is directly responsible for the clinical symptoms of our patients.
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Beatty, P., Ochs, H., Schreiber, R. et al. ABSENCE OF A MONOCLONAL ANTIBODY-DEFINED LEUKOCYTE PROTEIN IN PATIENTS WITH ABNORMAL LEUKOCYTE FUNCTION. Pediatr Res 18 (Suppl 4), 253 (1984). https://doi.org/10.1203/00006450-198404001-00958
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DOI: https://doi.org/10.1203/00006450-198404001-00958