Defective natural killer cytotoxicity and polymorphonuclear leukocyte antibody-dependent cellular cytotoxicity in patients with LFA-1/OKM-1 deficiency.
Four children with an immunodeficiency involving the absence of leukocyte membrane glycoproteins reacting with anti-LFA-1 and OKM-1 monoclonal antibodies were unable to mediate adherence-dependent leukocyte functions. Even with normal Fc receptor function, their PMN-ADCC and MC-NKC were markedly deficient. Single cell analysis demonstrated deficient antibody-mediated PMN-target cell adherence. Monoclonal antibodies against LFA-1 and OKM-1 reproduced this immunodeficiency in leukocytes from normal adults. LFA-1/OKM-1 mediates a PMN-target cell adhesive step.
D000293:Adolescent; D000911:Antibodies, Monoclonal / Q000502:physiology; D000920:Antibody-Dependent Cell Cytotoxicity; D000937:Antigen-Antibody Reactions; D000954:Antigens, Surface / Q000235:genetics* / Q000276:immunology; D002472:Cell Transformation, Viral; D002675:Child, Preschool; D003602:Cytotoxicity, Immunologic; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D007223:Infant; D007694:Killer Cells, Natural / Q000276:immunology*; D016169:Lymphocyte Function-Associated Antigen-1; D008297:Male; D008565:Membrane Proteins / Q000276:immunology; D009504:Neutrophils / Q000276:immunology*; D018139:Simplexvirus / Q000276:immunology
D000911:Antibodies, Monoclonal; D000954:Antigens, Surface; D016169:Lymphocyte Function-Associated Antigen-1; D008565:Membrane Proteins