LFA-1 IMMUNODEFICIENCY DISEASE - DEFINITION OF THE GENETIC-DEFECT AND CHROMOSOMAL MAPPING OF ALPHA-SUBUNIT AND BETA-SUBUNIT OF THE LYMPHOCYTE FUNCTION ASSOCIATED ANTIGEN-1 (LFA-1) BY COMPLEMENTATION IN HYBRID-CELLS
LFA-1 IMMUNODEFICIENCY DISEASE - DEFINITION OF THE GENETIC-DEFECT AND CHROMOSOMAL MAPPING OF ALPHA-SUBUNIT AND BETA-SUBUNIT OF THE LYMPHOCYTE FUNCTION ASSOCIATED ANTIGEN-1 (LFA-1) BY COMPLEMENTATION IN HYBRID-CELLS
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DOI：
10.1084/jem.164.3.855
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发表时间：
1986-09-01
影响因子：
15.3
通讯作者：
SPRINGER, TA
中科院分区：
文献类型：
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作者：
MARLIN, SD;MORTON, CC;SPRINGER, TA
淋巴细胞功能相关抗原1 （LFA-1）是一种白细胞粘附蛋白。我们研究了一种新的人类免疫缺陷疾病，其中LFA-1和另外两种具有相同β的蛋白质。白细胞表面缺乏亚基。遗传缺陷的基础是细胞表面表达的。和度量。通过LFA-1+小鼠T细胞系与患者或正常人细胞的体细胞融合来测定LFA-1的亚单位。人类LFA-1。和度量。来自正常细胞的亚基可以与小鼠LFA-1亚基结合形成种间杂交。复合物。的表面表达式。但不是。beta。病人细胞的亚单位被种间复合体的形成所拯救。这些发现表明LFA-1。基因缺陷细胞中的亚基在适当的。β存在时能胜任表面表达。亚基，表明基因损伤影响。亚基。人类LFA-1 α。和度量。亚基分别定位到16号和21号染色体上。据推测，遗传缺陷在21号染色体上。
Lymphocyte function associated antigen 1 (LFA-1) is a leukocyte cell adhesion protein. We have studied a novel human immunodeficiency disease in which LFA-1 and two other proteins which share the same .beta. subunit are lacking from the surface of leukocytes. The basis of the inherited defect in cell surface expression of both the .alpha. and .beta. subunits of LFA-1 was determined by somatic cell fusion of patient or normal human cells with an LFA-1+ mouse T cell line. Human LFA-1 .alpha. and .beta. subunits from normal cells could associate with mouse LFA-1 subunits to form interspecies hybrid .alpha..beta. complexes. Surface expression of the .alpha. but not the .beta. subunit of patient cells was rescued by the formation of interspecies complexes. These findings show that the LFA-1 .alpha. subunit in genetically deficient cells is competent for surface expression in the presence of an appropriate .beta. subunit, and suggest that the genetic lesion affects the .beta. subunit. The human LFA-1 .alpha. and .beta. subunits were mapped to chromosomes 16 and 21, respectively. The genetic defect is inferred to be on chromosome 21.