Adenosine-Deaminase Deficiency and Combined Immunodeficiency Syndrome

Dosagem sérica de adenosina deaminase em lúpus eritematoso sistêmico: ausência de associação com atividade de doença

“…A participação da adenosina deaminase (ADA) no sistema imunológico foi inicialmente sugerida pela verificação de imunodeficiência grave em portador da deficiência desta enzima (1) . Posteriormente, demonstrou-se sua participação na maturação de monócitos (2) e linfócitos (3,4) .…”

Correlation of adenosine deaminase activity with cell surface markers in acute lymphoblastic leukemia.

“…Tissue distribution studies show that ADA is usually present in all the tissues of the body with the highest activity in lymphocytes (3) and thymocytes (4). Furthermore, congenital absence ofthis enzyme, which arises as an inborn error ofmetabolism, results in the syndrome of severe combined immunodeficiency (5)(6)(7)(8). ADA activity has been found to be subnormal in the lymphocytes of patients with chronic lymphocytic leukemia (9, 10, 11) a disease characterized by immunologic impairment and an abnormal proliferation of B cells (12).…”

Adenosine deaminase and adenosine kinase in rat hepatomas and kidney tumours

“…In other systems there is experimental evidence suggesting that activity of adenosine deaminase (EC 3.5.4.4, ADA), which converts adenosine to inosine, may correlate in some way with cell proliferation. Congenital deficiency of ADA is associated with the severe combined immunodeficiency syndrome (Giblett et al, 1972;Dissing and Knudsen, 1972;Parkman et al, 1975). In peripheral lymphoid cells from patients with chronic lymphocytic leukaemia, ADA levels were in the normal range (Scholar and Calabresi, 1973), but blast cells from patients with acute lymphoblastic leukaemia and acute myeloid leukaemia contained markedly higher ADA activity than normal peripheral leucocytes (Smyth and Harrap, 1975;Meier, Coleman and Hutton, 1976).…”