Abnormal platelet aggregation in severe combined immunodeficiency disease with adenosine deaminase deficiency.
The platelets from a boy with adenosine deaminase deficiency-severe combined immunodeficiency disease (ADA-SCID) showed markedly subnormal aggregation in response to collagen and ADP. In contrast with normal platelets, ATP, AMP and adenosine had little effect in inhibiting ADP-induced aggregation of ADA-SCID platelets. These observations suggest that altered adenosine metabolism exists in ADA-SCID platelets.
D000243:Adenosine Deaminase / Q000172:deficiency*; D000244:Adenosine Diphosphate / Q000494:pharmacology; D003094:Collagen / Q000494:pharmacology; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000097:blood* / Q000201:enzymology; D007223:Infant; D008297:Male; D009700:Nucleoside Deaminases / Q000172:deficiency*; D010974:Platelet Aggregation / Q000187:drug effects
D000244:Adenosine Diphosphate; D003094:Collagen; D009700:Nucleoside Deaminases; D000243:Adenosine Deaminase