[HTML][HTML] The use of enzyme therapy to regulate the metabolic and phenotypic consequences of adenosine deaminase deficiency in mice: differential impact on …
MR Blackburn, M Aldrich, JB Volmer, W Chen… - Journal of Biological …, 2000 - ASBMB
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. ADA enzyme
therapy was used to examine the relative impact of ADA substrate elevations on these …
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. ADA enzyme
therapy was used to examine the relative impact of ADA substrate elevations on these …