Abstract
Chen et al. have reported that bone marrow transplantation in a patient with adenosine deaminase (ADA) deficiency and severe combined immunodeficiency is followed by clearing of one abnormal metabolite, deoxy(d)ATP, from the patient’s own still ADA-deficient erythrocytes (1). This finding suggests that engraftment of donor lymphocytes into an ADA-deficient patient provided “enzyme replacement therapy”, and completely corrected the accumulation of adenine deoxyribonucleotides in one of nonlymphoid tissues.
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© 1989 Plenum Press, New York
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Hyodo, Y., Itoh, R., Kurozumi, H., Ibe, M., Oka, J., Yamada, K. (1989). Immunological and Metabolic Reconstitution Following Successful Bone Marrow Transplantation from a HLA-Identical Sibling in an Infant with Adenosine Deaminase Deficeincy and Severe Combined Immunodeficiency: Partial Restoration of Purine Metabolism. In: Mikanagi, K., Nishioka, K., Kelley, W.N. (eds) Purine and Pyrimidine Metabolism in Man VI. Advances in Experimental Medicine and Biology, vol 253A. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-5673-8_88
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DOI: https://doi.org/10.1007/978-1-4684-5673-8_88
Publisher Name: Springer, Boston, MA
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