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Stem Cells Transl Med. 2013 Feb;2(2):112-7. doi: 10.5966/sctm.2012-0046. Epub 2013 Jan 22.

Elevated nuclear factor erythroid-2 levels promote epo-independent erythroid maturation and recapitulate the hematopoietic stem cell and common myeloid progenitor expansion observed in polycythemia vera patients.

Author information

1
Department of Hematology/Oncology, University Hospital Freiburg, Center for Clinical Research, Freiburg, Germany.

Abstract

The molecular etiology of polycythemia vera (PV) remains incompletely understood. Patients harbor increased numbers of hematopoietic stem cells and display Epo-independent erythroid maturation. However, the molecular mechanism underlying Epo hypersensitivity and stem cell expansion is unclear. We have previously shown that the transcription factor nuclear factor erythroid-2 (NF-E2) is overexpressed in the majority of PV patients. Here we demonstrated that elevation of NF-E2 expression in healthy CD34(+) cells to levels observed in PV caused Epo-independent erythroid maturation and expansion of hematopoietic stem cell (HSC) and common myeloid progenitor (CMP) cell numbers. Silencing NF-E2 in PV patients reverted both aberrancies, demonstrating for the first time that NF-E2 overexpression is both required and sufficient for Epo independence and HSC/CMP expansion in PV.

PMID:
23341442
PMCID:
PMC3659758
DOI:
10.5966/sctm.2012-0046
[Indexed for MEDLINE]
Free PMC Article

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