期刊
CELL
卷 135, 期 1, 页码 37-48出版社
CELL PRESS
DOI: 10.1016/j.cell.2008.09.016
关键词
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资金
- National Institutes of Health (NIH) [AI072571]
- Sandler Program for Asthma Research
- American Society of Hematology Junior Faculty Scholar
- NIH [AI007525, HD055165, GM007367, AI080184, AI078869]
- Paul and Daisy Soros Fellowship for New Americans
- Deutsche Forschungsgemeinschaft
- Howard Hughes Medical Institute
Plasmacytoid dendritic cells (PDCs) represent a unique immune cell type specialized in type I interferon (IFN) secretion in response to viral nucleic acids. The molecular control of PDC lineage specification has been poorly understood. We report that basic helix-loop-helix transcription factor (E protein) E2-2/Tcf4 is preferentially expressed in murine and human PDCs. Constitutive or inducible deletion of murine E2-2 blocked the development of PDCs but not of other lineages and abolished IFN response to unmethylated DNA. Moreover, E2-2 haploinsufficiency in mice and in human Pitt-Hopkins syndrome patients was associated with aberrant expression profile and impaired IFN response of the PDC. E2-2 directly activated multiple PDC-enriched genes, including transcription factors involved in PDC development (SpiB, Irf8) and function (Irf7). These results identify E2-2 as a specific transcriptional regulator of the PDC lineage in mice and humans and reveal a key function of E proteins in the innate immune system.
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