Immunodeficiency and chronic myelogenous leukemia-like syndrome in mice with a targeted mutation of the ICSBP gene.
作者： Thomas Holtschke , Jürgen Löhler , Yuka Kanno , Thomas Fehr , Nathalia Giese
DOI: 10.1016/S0092-8674(00)81348-3
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摘要: Abstract Interferon consensus sequence binding protein (ICSBP) is a transcription factor of the interferon (IFN) regulatory (IRF) family. Mice with null mutation ICSBP exhibit two prominent phenotypes related to previously described activities IRF The first enhanced susceptibility virus infections associated impaired production IFNγ. second deregulated hematopoiesis in both −/− and +/− mice that manifests as syndrome similar human chronic myelogenous leukemia. period disease progresses fatal blast crisis characterized by clonal expansion undifferentiated cells. Normal injected cells from developed acute leukemia within 6 weeks transfer. These results suggest novel role for regulating proliferation differentiation hematopoietic progenitor
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