Title: Metabolic abnormalities of human adenosine deaminase deficiency reproduced in the mouse by 2′-deoxycoformycin, an adenosine deaminase inhibitor
Abstract: Abstract Genetic absence of the enzyme adenosine deaminase in man results in severe combined immunodeficiency disease. Administration of 2′-deoxycoformycin, a tight-binding specific inhibitor of adenosine deaminase, to the mouse in vivo reproduces the metabolic abnormalities reported thus far in patients genetically lacking adenosine deaminase activity. These include markedly elevated excretion of 2′-deoxyadenosine, and inhibition of adenosine deaminase as well as of S-adenosyl homocysteine hydrolase tissue activities. Additionally there is a thymus-specific 10-fold increase in 2′-deoxyadenosine triphosphate content. This murine biochemical model of human adenosine deaminase deficiency should prove of value in studying the relationship of nucleoside metabolism to immunologic functional activity.
Publication Year: 1981
Publication Date: 1981-10-01
Language: en
Type: article
Indexed In: ['crossref', 'pubmed']
Access and Citation
Cited By Count: 19
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