Adenosine deaminase activity in recipients of bone marrow from immunodeficient mice homozygous for the wasted mutation.
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 marrow from wst/wst and littermate control mice into lethally irradiated normal recipients. The Vmax and Km values for ADA in recipient's hematologic and non-hematologic tissues did not differ significantly. These results indicate that the wasted mouse is not a model for ADA deficiency and SCID.
Willis
E H
EH
Carson
D A
DA
Shultz
L D
LD
D000243:Adenosine Deaminase / Q000172:deficiency / Q000378:metabolism*; D000818:Animals; D016026:Bone Marrow Transplantation; D006720:Homozygote; D007153:Immunologic Deficiency Syndromes / Q000201:enzymology*; D051379:Mice; D008812:Mice, Hairless; D008810:Mice, Inbred C57BL; D008817:Mice, Mutant Strains; D009154:Mutation; D009700:Nucleoside Deaminases / Q000378:metabolism*; D014018:Tissue Distribution
D009700:Nucleoside Deaminases; D000243:Adenosine Deaminase