Familial defect in the surface expression of the T-cell receptor-CD3 complex.
Familial defect in the surface expression of the T-cell receptor-CD3 complex.
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T 细胞受体-CD3 复合物表面表达的家族性缺陷。
DOI：
10.1056/nejm198811033191806
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发表时间：
1988
期刊：
影响因子：
--
通讯作者：
Terhorst,C
中科院分区：
文献类型：
--
作者：
Alarcon,B;Regueiro,JR;Arnaiz-Villena,A;Terhorst,C
患有严重联合免疫缺陷的婴儿通常在出生后的头几个月就会生病。尽管在几个月的时间里生长发育正常，但身高和体重的增长停止了，无法茁壮成长成为这种疾病的一个显著特征。大多数受影响的婴儿有严重的淋巴细胞减少症，并伴有成熟t细胞亚群的特异性缺陷。尽管严重联合免疫缺陷患者具有共同的临床表现，但潜在的t细胞缺陷是异质性的。严重联合免疫缺陷的一种亚型与胸腺细胞表达任何胸腺特异性抗原（CD3、CD4、CD6和CD8）的缺失有关。在……
INFANTS with severe combined immunodeficiency usually become ill in the first few months of life.1Although growth and development proceed normally for a few months, length and weight gain cease, and failure to thrive becomes a striking feature of the disorder.1Most affected infants have profound lymphopenia with a specific deficiency in the mature T-cell subset. Although patients with severe combined immunodeficiency share a common clinical picture, the underlying T-cell deficit is heterogeneous. One subtype of severe combined immunodeficiency is associated with the absence of the development of thymocytes that express any thymus-specific antigens (CD3, CD4, CD6, and CD8). In . . .