MOTH-EATEN AND VIABLE MOTH-EATEN MICE HAVE MUTATIONS IN THE HEMATOPOIETIC-CELL PHOSPHATASE GENE
MOTH-EATEN AND VIABLE MOTH-EATEN MICE HAVE MUTATIONS IN THE HEMATOPOIETIC-CELL PHOSPHATASE GENE
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DOI：
10.1038/ng0693-124
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发表时间：
1993-06-01
期刊：
影响因子：
30.8
通讯作者：
TSUI, FWL
中科院分区：
文献类型：
--
作者：
TSUI, HW;SIMINOVITCH, KA;TSUI, FWL
具有隐性蛾蚀素（me）或等位基因活性蛾蚀素（mel）突变的小鼠表现出严重的自身免疫和免疫缺陷综合征。我们已经证明，这些小鼠的基本缺陷涉及编码造血细胞磷酸酶（HCP）的基因的损伤。因此，这些小鼠提供了极好的模型，用于研究磷酸酶在造血中的作用以及将造血受损与严重免疫缺陷和全身性自身免疫表达联系起来的遗传和细胞事件的性质。
Mice with the recessive motheaten (me) or the allelic viable motheaten (mel mutations express a severe autoimmune and immunodeficiency syndrome. We have shown that the basic defect in these mice involves lesions in the gene which encodes haematopoietic cell phosphatase (HCP). These mice thus provide excellent models for investigating the roles of phosphatases in haematopoiesis and the nature of the genetic and cellular events linking impaired haematopoiesis to severe immunodeficiency and expression of systemic autoimmunity.