Article
The use of enzyme therapy to regulate the metabolic and phenotypic consequences of adenosine deaminase deficiency in mice. Differential impact on pulmonary and immunologic abnormalities.
The Journal of biological chemistry - 13 Oct 2000
Blackburn M R, Aldrich M, Volmer J B, Chen W, Zhong H, Kelly S, Hershfield M S, Datta S K, Kellems R E
Abstract excerpt
Adenosine deaminase (ADA) deficiency results in a combined immunodeficiency brought about by the immunotoxic properties of elevated ADA substrates. Additional non-lymphoid abnormalities are associated with ADA deficiency, however, little is known about how these relate to the metabolic consequences of ADA deficiency. ADA-deficient mice develop a combined immunodeficiency as well as severe pulmonary insufficiency....
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