Nucleotide Levels and Metabolism of Adenosine and Deoxyadenosine in Intact Erythrocytes Deficient in Adenosine Deaminase
We previously reported severe ATP depletion in the erythrocytes of an adenosine deaminase (ADA: EC 188.8.131.52) deficient child and also found raised dAMP levels1 in addition to the raised dATP and dADP levels noted by others.2 Post-marrow graft ATP levels returned to normal and the three deoxyadenosine nucleotides rapidly 2 disappeared. No ATP depletion had been noted in earlier reports. Similar findings have now been obtained in the erythrocytes of two further untreated ADA deficient children. Enzyme replacement therapy also resulted in a rapid return of nucleotide levels toward normal.3
KeywordsHigh Pressure Liquid Chromatography Enzyme Replacement Therapy Adenosine Deaminase Nucleotide Level Purine Nucleoside Phosphorylase
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- 2.Various authors reviewed in: Enzyme Defects and Immune Dysfunction Ciba Symposium 68 (new series) Exerpta Medica, Amsterdam (1979).Google Scholar