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Suppression of E-protein activity interferes with the development of BCR-ABL-mediated myeloproliferative disease.


ABSTRACT: E-proteins are a class of helix-loop-helix (HLH) proteins, which play multiple roles throughout lymphoid development. The DNA binding activities of the E-proteins are regulated by a distinct class of antagonistic HLH proteins, named Id1-4. Here we demonstrate that Id2 deficient mice in a C57BL/6 genetic background exhibit increased cellularity in the granulocyte/myeloid progenitor compartment and show significantly higher numbers of maturing neutrophils. Within 6 months of age, Id2 deficient mice succumbed from overwhelming granulocytosis. The disease closely mimicked the distinctive features of human chronic myeloid leukemia: leukocytosis with maturing neutrophils, splenomegaly, hepatomegaly, and myeloid infiltration into peripheral tissues, including spleen, liver, and lungs. Strikingly, forced Id2 expression in murine bone marrow cells substantially delayed the onset of myeloproliferative disease (MPD). Collectively, these studies show that suppression of E-protein activity interferes with the development of BCR-ABL-mediated MPD.

SUBMITTER: Ko J 

PROVIDER: S-EPMC2529058 | biostudies-literature | 2008 Sep

REPOSITORIES: biostudies-literature

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Suppression of E-protein activity interferes with the development of BCR-ABL-mediated myeloproliferative disease.

Ko Jinkyung J   Patel Nihal N   Ikawa Tomokatsu T   Kawamoto Hiroshi H   Frank Oliver O   Rivera Richard R RR   Van Etten Richard A RA   Murre Cornelis C  

Proceedings of the National Academy of Sciences of the United States of America 20080825 35


E-proteins are a class of helix-loop-helix (HLH) proteins, which play multiple roles throughout lymphoid development. The DNA binding activities of the E-proteins are regulated by a distinct class of antagonistic HLH proteins, named Id1-4. Here we demonstrate that Id2 deficient mice in a C57BL/6 genetic background exhibit increased cellularity in the granulocyte/myeloid progenitor compartment and show significantly higher numbers of maturing neutrophils. Within 6 months of age, Id2 deficient mic  ...[more]

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