concanavalin-a and Purine-Pyrimidine-Metabolism--Inborn-Errors

We have investigated a new hypothesis for the association between adenosine deaminase (A.D.A.) deficiency and immunodeficiency--namely, that deoxyadenosine rather than adenosine (an equally effective A.D.A. substrate) is toxic to proliferating cells of lymphoid origin. This possibility was explored in mitogen-stimulated lymphocytes cultured with a potent A.D.A. inhibitor, E.H.N.A. (erythro-9[2-hydroxy-3-nonyl] adenine) to simulate A.D.A. deficiency. In this in-vitro system deoxyadenosine was inhibitory at much lower and more physiological concentrations (1 mumol/1), compared with adenosine (100 mumol/1).

Topics: Adenosine; Adenosine Deaminase; Adenosine Deaminase Inhibitors; Cell Survival; Cells, Cultured; Child; Concanavalin A; Cytotoxicity, Immunologic; Deoxyadenosines; Enzyme Inhibitors; Humans; Immunologic Deficiency Syndromes; In Vitro Techniques; Lectins; Lymphocyte Activation; Lymphocytes; Mitogens; Nucleoside Deaminases; Purine-Pyrimidine Metabolism, Inborn Errors