Impaired granulopoiesis, myelodysplasia, and early lethality in CCAAT/enhancer binding protein ɛ-deficient mice

Polymorphonuclear leukocytes are essential for host defense to infectious diseases. CCAAT/enhancer binding protein ɛ (C/EBPɛ) is preferentially expressed in granulocytes and lymphoid cells. Mice with a null mutation in C/EBPɛ develop normally and are fertile but fail to generate functional neutrophi...

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Published in:Proceedings of the National Academy of Sciences - PNAS 1997-11, Vol.94 (24), p.13187-13192
Main Authors: Yamanaka, Ryuya, Barlow, Carrolee, Lekstrom-Himes, Julie, Castilla, Lucio H., Liu, Pu P., Eckhaus, Michael, Decker, Thomas, Wynshaw-Boris, Anthony, Xanthopoulos, Kleanthis G.
Format: Article
Language:English
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Biological Sciences
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Summary:Polymorphonuclear leukocytes are essential for host defense to infectious diseases. CCAAT/enhancer binding protein ɛ (C/EBPɛ) is preferentially expressed in granulocytes and lymphoid cells. Mice with a null mutation in C/EBPɛ develop normally and are fertile but fail to generate functional neutrophils and eosinophils. Opportunistic infections and tissue destruction lead to death by 3–5 months of age. Furthermore, end-stage mice develop myelodysplasia, characterized by proliferation of atypical granulocytes that efface the bone marrow and result in severe tissue destruction. Thus, C/EBPɛ is essential for terminal differentiation and functional maturation of committed granulocyte progenitor cells.
ISSN:0027-8424
1091-6490
DOI:10.1073/pnas.94.24.13187