Abstract
Children with the Chediak-Higashi (CH) syndrome are known to have abnormalities of natural killer (NK) cell function. We used the HNK-1 monoclonal antibody that reacts specifically with human NK and K cells to distinguish whether this abnormality was due either to a numerical deficiency of NK cells or a defect in their ability to function. In eight 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 five children with CH syndrome was higher than for age-matched normal controls (15.8% vs. 5.8%, P less than 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.
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- Abo T., Balch C. M. A differentiation antigen of human NK and K cells identified by a monoclonal antibody (HNK-1). J Immunol. 1981 Sep;127(3):1024–1029. [PubMed] [Google Scholar]
- Abo T., Cooper M. D., Balch C. M. Postnatal expansion of the natural killer and keller cell population in humans identified by the monoclonal HNK-1 antibody. J Exp Med. 1982 Jan 1;155(1):321–326. doi: 10.1084/jem.155.1.321. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Dent P. B., Fish L. A., White L. G., Good R. A. Chediak-Higashi syndrome. Observations on the nature of the associated malignancy. Lab Invest. 1966 Oct;15(10):1634–1642. [PubMed] [Google Scholar]
- Haliotis T., Roder J., Klein M., Ortaldo J., Fauci A. S., Herberman R. B. Chédiak-Higashi gene in humans I. Impairment of natural-killer function. J Exp Med. 1980 May 1;151(5):1039–1048. doi: 10.1084/jem.151.5.1039. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Klein M., Roder J., Haliotis T., Korec S., Jett J. R., Herberman R. B., Katz P., Fauci A. S. Chédiak-Higashi gene in humans. II. The selectivity of the defect in natural-killer and antibody-dependent cell-mediated cytotoxicity function. J Exp Med. 1980 May 1;151(5):1049–1058. doi: 10.1084/jem.151.5.1049. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Prchal J. T., Crist W. M., Malluh A., Vitek A., Tauxe W. N., Carroll A. J. A new glucose-6-phosphate dehydrogenase deficient variant in a patient with Chediak-Higashi syndrome. Blood. 1980 Sep;56(3):476–480. [PubMed] [Google Scholar]
- Roder J. C., Haliotis T., Klein M., Korec S., Jett J. R., Ortaldo J., Heberman R. B., Katz P., Fauci A. S. A new immunodeficiency disorder in humans involving NK cells. Nature. 1980 Apr 10;284(5756):553–555. doi: 10.1038/284553a0. [DOI] [PubMed] [Google Scholar]
- Timonen T., Saksela E. Isolation of human NK cells by density gradient centrifugation. J Immunol Methods. 1980;36(3-4):285–291. doi: 10.1016/0022-1759(80)90133-7. [DOI] [PubMed] [Google Scholar]
- White J. G., Clawson C. C. The Chediak-Higashi syndrome: microtubules in monocytes and lymphocytes. Am J Hematol. 1979;7(4):349–356. doi: 10.1002/ajh.2830070407. [DOI] [PubMed] [Google Scholar]
- Windhorst D. B., Padgett G. The Chediak-Higashi syndrome and the homologous trait in animals. J Invest Dermatol. 1973 Jun;60(6):529–537. doi: 10.1111/1523-1747.ep12703609. [DOI] [PubMed] [Google Scholar]