Increased purine nucleotides in adenosine deaminase-deficient lymphocytes.
It was found that ATP and cyclic AMP were greatly increased in human blood lymphocytes which were deficient in ADA. Certain other purine and pyrimidine nucleotides were elevated but to a lesser degree. Energy production in these cells may be inhibited by the increase in nucleotides since the ATP:ADP ratio was significantly below normal. Thus it appears that the immunologic deficiency in human ADA deficiency is related to increased nucleotide concentrations in the lymphocytes.
D000243:Adenosine Deaminase / Q000032:analysis / Q000172:deficiency*; D000244:Adenosine Diphosphate / Q000032:analysis; D000255:Adenosine Triphosphate / Q000032:analysis; D000242:Cyclic AMP / Q000032:analysis / Q000096:biosynthesis; D006160:Guanosine Triphosphate / Q000032:analysis; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000378:metabolism; D007223:Infant; D008214:Lymphocytes / Q000378:metabolism*; D008297:Male; D009700:Nucleoside Deaminases / Q000172:deficiency*; D011687:Purines / Q000032:analysis* / Q000096:biosynthesis; D011742:Pyrimidine Nucleotides / Q000032:analysis* / Q000096:biosynthesis
D011687:Purines; D011742:Pyrimidine Nucleotides; D000244:Adenosine Diphosphate; D006160:Guanosine Triphosphate; D000255:Adenosine Triphosphate; D000242:Cyclic AMP; D009700:Nucleoside Deaminases; D000243:Adenosine Deaminase