dATP accumulation and ATP depletion in platelets in adenosine deaminase deficiency: significance for the immune response?
High levels of dATP and dADP, accompanied by ATP depletion, were found in the platelets of two ADA-deficient children with severe combined immunodeficiency (SCID). In vitro studies demonstrated that even normal platelets had the ability to make dATP from deoxyadenosine (dAR) under physiological conditions. This capability was greatly enhanced by conditions simulating ADA deficiency. These results question whether the platelet has a specific role in the normal immune response.
D000243:Adenosine Deaminase / Q000172:deficiency*; D000255:Adenosine Triphosphate / Q000097:blood*; D001792:Blood Platelets / Q000201:enzymology / Q000378:metabolism*; D003070:Coformycin / Q000031:analogs & derivatives / Q000494:pharmacology; D003838:Deoxyadenine Nucleotides / Q000097:blood*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000097:blood*; D009700:Nucleoside Deaminases / Q000172:deficiency*; D009711:Nucleotides / Q000097:blood; D015649:Pentostatin
D003838:Deoxyadenine Nucleotides; D009711:Nucleotides; D003070:Coformycin; D015649:Pentostatin; D000255:Adenosine Triphosphate; D009700:Nucleoside Deaminases; D000243:Adenosine Deaminase; C026600:2'-deoxyadenosine triphosphate