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

Authors: Chiba, S  Takahashi, T  Takeshita, K  Minowada, J  Yazaki, Y  Ruddle, FH  Hirai, H 
Citation: Chiba S, etal., Blood. 1997 Jul 1;90(1):97-104.
Pubmed: (View Article at PubMed) PMID:9207443

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.

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CRRD ID: 11041603
Created: 2016-03-24
Species: All species
Last Modified: 2016-03-24
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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.