The importance of adenosine deaminase for lymphocyte development and function
The importance of adenosine deaminase for lymphocyte development and function
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DOI：
10.1006/bbrc.2000.2773
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发表时间：
2000-06-07
影响因子：
3.1
通讯作者：
Kellems, RE
中科院分区：
文献类型：
--
作者：
Aldrich, MB;Blackburn, MR;Kellems, RE
人体腺苷脱氨酶（ADA）缺乏主要表现为严重的淋巴细胞减少症和免疫缺陷，如果不治疗，会导致6个月龄时的死亡。在这篇综述中，我们讨论了疾病的表型，生化和代谢特征，并描述了一种小鼠模型，在该模型中，ADA的水平可以被生化和遗传操纵。这个模型为揭示嘌呤分解代谢酶影响淋巴细胞生成的机制提供了令人兴奋的可能性。(C)北京大学出版社.
Deficiency in the enzyme adenosine deaminase (ADA) in humans manifests primarily as severe lymphopenia and immunodeficiency, resulting in death by 6 months of age, if untreated. In this review, we discuss phenotypical, biochemical, and metabolic hallmarks of the disease, and describe a mouse model in which levels of ADA can be biochemically and genetically manipulated. This model provides exciting possibilities for uncovering the mechanisms by which this purine catabolic enzyme affects lymphopoiesis. (C) 2000 Academic Press.