Abstract
Mice homozygous for the mutation wasted (wst/wst) have been postulated to be a model for the form of human severe combined immunodeficiency disease (SCID) that is secondary to a genetic deficiency of adenosine deaminase (ADA). To test this hypothesis more critically, we transplanted raarrrow from wst/wst and littermate control mice into lethally irradiated normal recipients. If there was an inherent ADA deficiency in the hematopoietic stem cells of the wst/wst mutant, the defect would be expected to be passed on to the irradiated marrow chimeras, resulting in decreased Vmax values for ADA, and/or changes in the Km for adenosine. However, the Vmax and Km values for ADA and adenosine, repsectively, in recipient's hematologic and non-hematologic tissues did not differ significantly from control values. In addition, we also found no significant differences in ADA activities between lymphoid and liver tissue from untreated 24-to-28-day-old wst/wst and littermate control mice. These results indicate that the wasted mouse is not a model for ADA deficiency and SCID.
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Willts, E., Shultz, L. & Carson, D. 173 ADENOSINE DEAMINASE ACTIVITY IN RECIPIENTS OF BONE MARROU FROM IMMUNODEFICIENT MICE HOMOZYGOUS FOR THE WASTED MUTATION. Pediatr Res 24, 140 (1988). https://doi.org/10.1203/00006450-198807000-00197
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DOI: https://doi.org/10.1203/00006450-198807000-00197