Hyperactivation and Proliferation of Lymphocytes from the Spleens of Flaky Skin (fsn) Mutant Mice

Mice homozygous for the flaky skin (fsn) single gene mutation have a severe hyperproliferative disease resulting in a complex phenotype, which includes widespread inflammation and autoimmunity. This study sought to characterize lymphocyte function of flaky skin mutant mice. Flaky skin lymphocytes sh...

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Bibliographic Details
Published in:Autoimmunity (Chur, Switzerland) Switzerland), 2004-05, Vol.37 (3), p.227-235
Main Authors: Welner, Robert, Hastings, William, Hill, Beth L., Pelsue, Stephen C.
Format: Article
Language:English
Subjects:
Animals
Autoimmunity
B-Lymphocytes - metabolism
Biological and medical sciences
Cell Proliferation
Fundamental and applied biological sciences. Psychology
Fundamental immunology
General aspects
Immunopathology
Interleukin-2 - metabolism
Interleukin-4
Interleukin-4 - metabolism
Interleukin-7 - metabolism
Lymphocyte Activation - physiology
Medical sciences
Mice
Mice, Mutant Strains
Mouse model
Phenotype
Spleen
STAT6
STAT6 Transcription Factor
T-Lymphocytes - metabolism
Trans-Activators - metabolism
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Summary:Mice homozygous for the flaky skin (fsn) single gene mutation have a severe hyperproliferative disease resulting in a complex phenotype, which includes widespread inflammation and autoimmunity. This study sought to characterize lymphocyte function of flaky skin mutant mice. Flaky skin lymphocytes show enhanced proliferation with in vitro mitogen stimulated spleen cells, as well as enriched splenic B- and T-cells. The production of IL-4 by fsn/fsn T-lymphocytes is increased dramatically compared with normal controls. Flaky skin lymphocytes exhibited increased responsiveness to IL-2, IL-4 and IL-7 in the absence of pre-activation, enhanced IgE production in response to ovalbumin immunization, and constitutive STAT6 activation. These data indicate that the cytokines IL-2, IL-4 and IL-7 likely contribute to the lymphocyte activation in fsn/fsn mutant mice. This lymphocyte hyperactivation may result in the development of systemic autoimmunity in fsn/fsn mutant mice.
ISSN:0891-6934
1607-842X
DOI:10.1080/08916930410001666659