Chediak‐higashi syndrome derived t cell lines manifest giant lysosomal granules, normal natural killer cell, and lectin‐mediated cytotoxicity
作者:
Fernando Merino,
Michaeleen Collins,
David T. Purtilo,
期刊:
Journal of Clinical Laboratory Analysis
(WILEY Available online 1987)
卷期:
Volume 1,
issue 1
页码: 72-76
ISSN:0887-8013
年代: 1987
DOI:10.1002/jcla.1860010111
出版商: Wiley Subscription Services, Inc., A Wiley Company
关键词: Chediak–Higashi syndrome;T cells;Epstein–Barr virus;natural killer cell activity;lysosomal granules
数据来源: WILEY
摘要:
AbstractLymphoblastoid T cell lines from patients with Chediak‐Higashi syndrome (CHS) have been established by stimulating lymphocytes with phytohemagglutinin and interleukin‐2. This culture technique produced T cells containing giant granules which are characteristically seen in peripheral blood leukocytes of children with the disease. The granules were lysosomes as demonstrated by their reactivity with acid phosphatase. Functionally, they exhibited normal natural killer cell activity and/or lectin‐induced cytotoxicity. Variations in function of freshly obtained and long‐term cultured T cells are discussed. These cell lines will permit further studies to elucidate defects in lymphoid cells derived from patients w
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