NATURAL-KILLER (HNK-1+) CELLS IN CHEDIAK-HIGASHI PATIENTS ARE PRESENT IN NORMAL NUMBERS BUT ARE ABNORMAL IN FUNCTION AND MORPHOLOGY
NATURAL-KILLER (HNK-1+) CELLS IN CHEDIAK-HIGASHI PATIENTS ARE PRESENT IN NORMAL NUMBERS BUT ARE ABNORMAL IN FUNCTION AND MORPHOLOGY
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DOI：
10.1172/jci110592
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发表时间：
1982-01-01
影响因子：
15.9
通讯作者：
BALCH, CM
中科院分区：
文献类型：
--
作者：
ABO, T;RODER, JC;BALCH, CM
切迪亚克-东(CH)综合征的儿童已知有自然杀伤(NK)细胞功能异常。使用与人类NK和K[杀手]细胞发生特异性反应的HNK-1单抗来区分这种异常是由于NK细胞数量不足还是其功能缺陷所致。在8例CH患者中，有相当比例(10-19%)的单个核细胞表达HNK-1分化抗原。5例CH综合征患儿NK细胞水平明显高于正常对照组(15.8%vs.5.8%，P<；0.001)。当用荧光激活细胞分选仪分离HNK-1+细胞时，CH患者的NK细胞是单一大颗粒的均一淋巴细胞群体，而不是正常人NK细胞中的多个小颗粒。来自CH患者的纯化的HNK-1+细胞具有最低的NK或K细胞功能。因此，CH综合征包括功能缺陷的NK细胞群体，这些细胞保留了表达HNK-1分化抗原的能力。
Children with the Chediak-Higashi (CH) syndrome are known to have abnormalities of natural killer (NK) cell function. The HNK-1 monoclonal antibody that reacts specifically with human NK and K [killer] cells was used to distinguish whether this abnormality was due either to a numerical deficiency of NK cells or a defect in their ability to function. In 8 CH patients, a significant proportion of their blood mononuclear cells (10-19%) expressed the HNK-1 differentiation antigen. The level of NK cells in the 5 children with CH syndrome was higher than for age-matched normal controls (15.8% vs. 5.8%, P < 0.001). When HNK-1+ cells were isolated with a fluorescence-activated cell sorter, the NK cells from CH patients were a homogeneous population of lymphocytes with a single large granule rather than the multiple small granules seen in NK cells from normal individuals. The purified HNK-1+ cells from the CH patients had minimal NK or K cell function. The CH syndrome thus includes a functionally defective population of NK cells that retain the capability of expressing the HNK-1 differentiation antigen.