Inosine Formation from Hypoxanthine by Intact Erythrocytes and Fibroblasts of an Immunodeficient Child with Purine Nucleoside Phosphorylase Deficiency
Part of the
Advances in Experimental Medicine and Biology
book series (AEMB, volume 165)
Detailed studies of purine metabolism have been carried out over an 18 month period in a neonate with severe T-cell immunodeficiency(Watson et al, this symposium). A complete deficiency of the enzyme purine nucleoside Phosphorylase (PNP: EC 126.96.36.199) had been found in lysed erythrocytes and leucocytes in both the degradative and synthetic direction (hypoxanthine to inosine). This paper reports in vitro studies using intact erythrocytes and fibroblasts from this child in which the formation of inosine from (8-14C) hypoxanthine was a consistent finding on separate occasions.
KeywordsPurine Metabolism Purine Nucleoside Phosphorylase Lysed Erythrocyte Intact Erythrocyte Physiological Phosphate
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