Biochemical study of a case of hemolytic anemia with increased (85 fold) red cell adenosine deaminase.
An increased red cell adenosine deaminase (ADA) activity (85-fold) was found in a 10-year-old male child suffering from severe hemolytic disease. Evidence is given that the excessive ADA activity in erythrocytes is due to an abnormal amount of a catalytically and immunologically normal enzyme. Metabolic studies with the patient's erythrocytes show that the low ATP concentration in these cells (64% of comparably reticulocyte-rich blood) is due both to a diminished synthesis of adenylic nucleotides from adenosine, and to an excessive catabolism of AMP.
D000241:Adenosine; D000243:Adenosine Deaminase / Q000378:metabolism*; D000249:Adenosine Monophosphate / Q000378:metabolism; D000255:Adenosine Triphosphate / Q000378:metabolism; D000745:Anemia, Hemolytic, Congenital / Q000201:enzymology*; D002648:Child; D004912:Erythrocytes / Q000201:enzymology / Q000378:metabolism; D006801:Humans; D008297:Male; D009700:Nucleoside Deaminases / Q000378:metabolism*
D000249:Adenosine Monophosphate; D000255:Adenosine Triphosphate; D009700:Nucleoside Deaminases; D000243:Adenosine Deaminase; D000241:Adenosine