Open AccessChediak‐higashi syndrome derived t cell lines manifest giant lysosomal granules, normal natural killer cell, and lectin‐mediated cytotoxicity
Author(s) -
Merino Fernando,
Collins Michaeleen,
Purtilo David T.
Publication year - 1987
Publication title -
journal of clinical laboratory analysis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.536
H-Index - 50
eISSN - 1098-2825
pISSN - 0887-8013
DOI - 10.1002/jcla.1860010111
Subject(s) - chédiak–higashi syndrome , cytotoxicity , acid phosphatase , lectin , natural killer cell , biology , immunology , cell culture , lymphokine activated killer cell , microbiology and biotechnology , chemistry , t cell , immune system , in vitro , interleukin 21 , biochemistry , enzyme , genetics
Abstract Lymphoblastoid 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 with CHS.