Treatment of Acute Lymphoblastic Leukemia with the Adenosine Deaminase Inhibitor 2′-Deoxycoformycin
Congenital deficiency of the enzyme adenosine deaminase (ADA, EC 18.104.22.168.) is associated with severe combined immunodeficiency disease characterized by marked lymphopenia and thymic involution.1 ADA catalyzes the deamination of adenosine and deoxyadenosine to inosine and deoxyinosine, respectively, and it has been proposed that the increased levels of deoxyadenosine associated with ADA deficiency lead to the selective accumulation of dATP by lymphoid cells, inhibition of DNA synthesis and cell death.2
KeywordsAcute Lymphoblastic Leukemia Adenosine Deaminase Thymic Involution Severe Combine Immunodeficiency Disease Adenosine Concentration
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