Perryman L E, Magnuson N S
Prog Clin Biol Res. 1982;94:271-307.
Significant contributions to understanding the role of lymphocyte subpopulations in the immune response and to the characterization of immunodeficiencies in children have been achieved through study of animal models of immunodeficiency. Additional contributions can be made in two important areas. One is through identification of relevant, naturally-occurring models of adenosine deaminase deficiency and purine nucleoside phosphorylase deficiency. The second, and potentially more important contribution, would be the identification of the metabolic basis for existing immune deficiencies. The necessary collaborative working arrangements should be established to achieve this objective. Demonstration of the metabolic basis for immune deficiencies in animals may enable the identification of similar defects in people, and eventually lead to enzyme or metabolite delivery schemes for treatment of affected children.
通过对免疫缺陷动物模型的研究，在理解淋巴细胞亚群在免疫反应中的作用以及儿童免疫缺陷的特征方面取得了重大进展。在两个重要领域还可以做出进一步贡献。一是通过识别腺苷脱氨酶缺乏症和嘌呤核苷磷酸化酶缺乏症相关的自然发生模型。第二个可能更重要的贡献是确定现有免疫缺陷的代谢基础。应建立必要的合作工作安排以实现这一目标。证明动物免疫缺陷的代谢基础可能有助于识别人类中的类似缺陷，并最终导致为受影响儿童制定酶或代谢物递送治疗方案。
