EHNA is a poor inhibitor of deoxyadenosine catabolism in cultured human lymphocytes.
Erythro-9-(2-hydroxy-3-nonyl)-adenine (EHNA) has been used by many workers as enzyme inhibitor in vitro to simulate the in vivo situation in inherited adenosine deaminase (ADA) deficiency. In this study the metabolism of 8-14C deoxyadenosine (dAR) has been followed in cultured lymphocytes from patients deficient in enzymes associated with the catabolism and salvage of dAR, in the absence and presence of 10 microM EHNA. The results show that EHNA, at these concentrations, does not prevent the catabolism of dAR and thus does not provide a valid model for investigating the toxicity to the immune system in inherited ADA deficiency.
D000225:Adenine / Q000031:analogs & derivatives* / Q000494:pharmacology; D000228:Adenine Phosphoribosyltransferase / Q000172:deficiency; D000243:Adenosine Deaminase / Q000172:deficiency / Q000378:metabolism; D058892:Adenosine Deaminase Inhibitors; D002460:Cell Line; D003839:Deoxyadenosines / Q000378:metabolism*; D004305:Dose-Response Relationship, Drug; D006801:Humans; D007041:Hypoxanthine Phosphoribosyltransferase / Q000172:deficiency; D007153:Immunologic Deficiency Syndromes / Q000235:genetics; D007700:Kinetics; D007926:Lesch-Nyhan Syndrome / Q000378:metabolism; D008214:Lymphocytes / Q000187:drug effects* / Q000378:metabolism; D009700:Nucleoside Deaminases / Q000037:antagonists & inhibitors*; D011683:Purine-Nucleoside Phosphorylase / Q000172:deficiency; D011686:Purine-Pyrimidine Metabolism, Inborn Errors / Q000201:enzymology* / Q000378:metabolism; D012107:Research Design
D058892:Adenosine Deaminase Inhibitors; D003839:Deoxyadenosines; C010192:9-(2-hydroxy-3-nonyl)adenine; D011683:Purine-Nucleoside Phosphorylase; D000228:Adenine Phosphoribosyltransferase; D007041:Hypoxanthine Phosphoribosyltransferase; D009700:Nucleoside Deaminases; D000243:Adenosine Deaminase; D000225:Adenine