The importance of adenosine deaminase for lymphocyte development and function

Deficiency in the enzyme adenosine deaminase (ADA) in humans manifests prim arily as severe lymphopenia and immunodeficiency, resulting in death by 6 m onths of age, if untreated. In this review, we discuss phenotypical, bioche mical, and metabolic hallmarks of the disease, and describe a mouse model i n which levels of ADA can be biochemically and genetically manipulated. Thi s model provides exciting possibilities for uncovering the mechanisms by wh ich this purine catabolic enzyme affects lymphopoiesis. (C) 2000 Academic P ress.