Selective expression of mRNA coding for the truncated form of erythropoietin receptor in hematopoietic cells and its decrease in patients with polycythemia vera

Shigeru Chiba, Tokiharu Takahashi, Kenichi Takeshita, Jun Minowada, Yoshio Yazaki, Frank H. Ruddle, Hisamaru Hirai

    Research output: Contribution to journalArticlepeer-review

    Abstract

    The mRNA encoding full-length erythropoietin (EPO) receptor (EPOR-F) comprises exons I through VIII. Another membrane-bound EPOR (EPOR-T) isoform has a truncated cytoplasmic region and is encoded by the mRNA containing unspliced intron VII (EPOR-T mRNA). EPOR-T is believed to have a dominantly negative function against EPOR-F. We show that EPOR-T mRNA is markedly decreased in the blood cells of patients with polycythemia vera (PV). We also show that EPOR-T mRNA is not detected in erythroid/megakaryocytic leukemia cell lines, but is expressed in nonerythroid/nonmegakaryocytic lines, suggesting the presence of a cell type-specific system by which intron VII of the EPOR transcript is spliced. Deregulation of this splicing system in early hematopoietic progenitors possibly explains the profound decrease in EPOR-T mRNA and consequent pathophysiology of PV.
    Original languageEnglish
    Pages (from-to)97-104
    Number of pages7
    JournalBlood
    Volume90
    Issue number1
    Publication statusPublished - 1 Jul 1997

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