author_facet Klein, M
Roder, J
Haliotis, T
Korec, S
JR, Jett
Herberman, RB
Katz, P
Fauci, AS
Klein, M
Roder, J
Haliotis, T
Korec, S
JR, Jett
Herberman, RB
Katz, P
Fauci, AS
author Klein, M
Roder, J
Haliotis, T
Korec, S
JR, Jett
Herberman, RB
Katz, P
Fauci, AS
spellingShingle Klein, M
Roder, J
Haliotis, T
Korec, S
JR, Jett
Herberman, RB
Katz, P
Fauci, AS
The Journal of Experimental Medicine
Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
Immunology
Immunology and Allergy
author_sort klein, m
spelling Klein, M Roder, J Haliotis, T Korec, S JR, Jett Herberman, RB Katz, P Fauci, AS 1540-9538 0022-1007 Rockefeller University Press Immunology Immunology and Allergy http://dx.doi.org/10.1084/jem.151.5.1049 <jats:p>Antibody-dependent cell-mediated cytolysis (ADCC) of human tumor cells by FcR(+) nonadherent effector lymphocytes as well as natural killer (NK) activity was markedly impaired in Chediak-Steinbrinck-Higashi Syndrome (C-HS) patients. Compared to a more than 400-fold defect in NK activity in terms of lytic units, the abnormal ADCC response in C-HS donors was 24-fold below normal suggesting a partial but not complete overlap of lymphocytes or lytic mechanisms responsible for ADCC and NK. The ADCC mechanism against erythrocyte targets, however, was normal, thereby suggesting a qualitative difference in these two forms of ADCC. Other effector-cell functions against tumor-cell targets were normal as measured by (a) spontaneous cytolysis mediated by monocytes, (b) spontaneous cytostasis mediated by neutrophils, and (c) lectin-dependent cytolysis mediated by neutrophils. Although one C-HS patient was low in lectin-dependent cytolysis mediated by lymphocytes, the other C-HS patient was normal, thereby suggesting that cytolytic T function was not linked to the NK-ADCC defect. In addition, the proliferative response to T-dependent mitogens was also relatively normal. These results, combined with other studies showing normal cell-mediated and humoral immunity in these same patients, suggest that patients with C-HS have an immunodeficiency which is selective for NK and ADCC activity.</jats:p> Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function The Journal of Experimental Medicine
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series The Journal of Experimental Medicine
source_id 49
title Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
title_unstemmed Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
title_full Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
title_fullStr Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
title_full_unstemmed Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
title_short Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
title_sort chediak-higashi gene in humans. ii. the selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
topic Immunology
Immunology and Allergy
url http://dx.doi.org/10.1084/jem.151.5.1049
publishDate 1980
physical 1049-1058
description <jats:p>Antibody-dependent cell-mediated cytolysis (ADCC) of human tumor cells by FcR(+) nonadherent effector lymphocytes as well as natural killer (NK) activity was markedly impaired in Chediak-Steinbrinck-Higashi Syndrome (C-HS) patients. Compared to a more than 400-fold defect in NK activity in terms of lytic units, the abnormal ADCC response in C-HS donors was 24-fold below normal suggesting a partial but not complete overlap of lymphocytes or lytic mechanisms responsible for ADCC and NK. The ADCC mechanism against erythrocyte targets, however, was normal, thereby suggesting a qualitative difference in these two forms of ADCC. Other effector-cell functions against tumor-cell targets were normal as measured by (a) spontaneous cytolysis mediated by monocytes, (b) spontaneous cytostasis mediated by neutrophils, and (c) lectin-dependent cytolysis mediated by neutrophils. Although one C-HS patient was low in lectin-dependent cytolysis mediated by lymphocytes, the other C-HS patient was normal, thereby suggesting that cytolytic T function was not linked to the NK-ADCC defect. In addition, the proliferative response to T-dependent mitogens was also relatively normal. These results, combined with other studies showing normal cell-mediated and humoral immunity in these same patients, suggest that patients with C-HS have an immunodeficiency which is selective for NK and ADCC activity.</jats:p>
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author Klein, M, Roder, J, Haliotis, T, Korec, S, JR, Jett, Herberman, RB, Katz, P, Fauci, AS
author_facet Klein, M, Roder, J, Haliotis, T, Korec, S, JR, Jett, Herberman, RB, Katz, P, Fauci, AS, Klein, M, Roder, J, Haliotis, T, Korec, S, JR, Jett, Herberman, RB, Katz, P, Fauci, AS
author_sort klein, m
container_issue 5
container_start_page 1049
container_title The Journal of Experimental Medicine
container_volume 151
description <jats:p>Antibody-dependent cell-mediated cytolysis (ADCC) of human tumor cells by FcR(+) nonadherent effector lymphocytes as well as natural killer (NK) activity was markedly impaired in Chediak-Steinbrinck-Higashi Syndrome (C-HS) patients. Compared to a more than 400-fold defect in NK activity in terms of lytic units, the abnormal ADCC response in C-HS donors was 24-fold below normal suggesting a partial but not complete overlap of lymphocytes or lytic mechanisms responsible for ADCC and NK. The ADCC mechanism against erythrocyte targets, however, was normal, thereby suggesting a qualitative difference in these two forms of ADCC. Other effector-cell functions against tumor-cell targets were normal as measured by (a) spontaneous cytolysis mediated by monocytes, (b) spontaneous cytostasis mediated by neutrophils, and (c) lectin-dependent cytolysis mediated by neutrophils. Although one C-HS patient was low in lectin-dependent cytolysis mediated by lymphocytes, the other C-HS patient was normal, thereby suggesting that cytolytic T function was not linked to the NK-ADCC defect. In addition, the proliferative response to T-dependent mitogens was also relatively normal. These results, combined with other studies showing normal cell-mediated and humoral immunity in these same patients, suggest that patients with C-HS have an immunodeficiency which is selective for NK and ADCC activity.</jats:p>
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imprint Rockefeller University Press, 1980
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spelling Klein, M Roder, J Haliotis, T Korec, S JR, Jett Herberman, RB Katz, P Fauci, AS 1540-9538 0022-1007 Rockefeller University Press Immunology Immunology and Allergy http://dx.doi.org/10.1084/jem.151.5.1049 <jats:p>Antibody-dependent cell-mediated cytolysis (ADCC) of human tumor cells by FcR(+) nonadherent effector lymphocytes as well as natural killer (NK) activity was markedly impaired in Chediak-Steinbrinck-Higashi Syndrome (C-HS) patients. Compared to a more than 400-fold defect in NK activity in terms of lytic units, the abnormal ADCC response in C-HS donors was 24-fold below normal suggesting a partial but not complete overlap of lymphocytes or lytic mechanisms responsible for ADCC and NK. The ADCC mechanism against erythrocyte targets, however, was normal, thereby suggesting a qualitative difference in these two forms of ADCC. Other effector-cell functions against tumor-cell targets were normal as measured by (a) spontaneous cytolysis mediated by monocytes, (b) spontaneous cytostasis mediated by neutrophils, and (c) lectin-dependent cytolysis mediated by neutrophils. Although one C-HS patient was low in lectin-dependent cytolysis mediated by lymphocytes, the other C-HS patient was normal, thereby suggesting that cytolytic T function was not linked to the NK-ADCC defect. In addition, the proliferative response to T-dependent mitogens was also relatively normal. These results, combined with other studies showing normal cell-mediated and humoral immunity in these same patients, suggest that patients with C-HS have an immunodeficiency which is selective for NK and ADCC activity.</jats:p> Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function The Journal of Experimental Medicine
spellingShingle Klein, M, Roder, J, Haliotis, T, Korec, S, JR, Jett, Herberman, RB, Katz, P, Fauci, AS, The Journal of Experimental Medicine, Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function, Immunology, Immunology and Allergy
title Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
title_full Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
title_fullStr Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
title_full_unstemmed Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
title_short Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
title_sort chediak-higashi gene in humans. ii. the selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
title_unstemmed Chediak-Higashi gene in humans. II. The selectivity of the defect in natural- killer and antibody-dependent cell-mediated cytotoxicity function
topic Immunology, Immunology and Allergy
url http://dx.doi.org/10.1084/jem.151.5.1049