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Publication : Hyperactivation and proliferation of lymphocytes from the spleens of flaky skin (fsn) mutant mice.

First Author  Welner R Year  2004
Journal  Autoimmunity Volume  37
Issue  3 Pages  227-35
PubMed ID  15497457 Mgi Jnum  J:102002
Mgi Id  MGI:3606384 Doi  10.1080/08916930410001666659
Citation  Welner R, et al. (2004) Hyperactivation and proliferation of lymphocytes from the spleens of flaky skin (fsn) mutant mice. Autoimmunity 37(3):227-35
abstractText  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.
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