Generation of transgenic mice expressing human hemoglobin E

Qiuying Chen, Eric E. Bouhassira, Arnaud Besse, Sandra M. Suzuka, Mary E. Fabry, Ronald L. Nagel, Rhoda Elison Hirsch

Research output: Contribution to journalArticle

10 Scopus citations

Abstract

Hemoglobin E (HbE, β26 Glu→Lys) is the most common abnormal Hb variant in the world, and found in greatest frequency in Southeast (SE) Asia. In the United States, HbE is the third most prevalent variant (after HbS and HbC); and its now increasing frequency is due to immigration from SE Asia. HbE homozygotes present a benign clinical picture, but when HbE is coupled with β 0-thalassemia or HbS, variably severe hemoglobinopathies arise. To date, there are no transgenic animal models of HbE-related diseases. We report here the creation of transgenic mice expressing human HbE as a step toward creating animal models for HbE-related diseases. The β E mice exhibit red blood cell hypochromia and target cells consistent with those observed in human patients exhibiting HbE trait. Furthermore, the transgenic HbE hemolysates contain increased amounts of Hb oxidation products.

Original languageEnglish (US)
Pages (from-to)303-307
Number of pages5
JournalBlood Cells, Molecules, and Diseases
Volume33
Issue number3
DOIs
StatePublished - Nov 1 2004

Keywords

  • Hemoglobin E
  • Homozygote
  • Transgenic

ASJC Scopus subject areas

  • Molecular Medicine
  • Molecular Biology
  • Hematology
  • Cell Biology

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    Chen, Q., Bouhassira, E. E., Besse, A., Suzuka, S. M., Fabry, M. E., Nagel, R. L., & Hirsch, R. E. (2004). Generation of transgenic mice expressing human hemoglobin E. Blood Cells, Molecules, and Diseases, 33(3), 303-307. https://doi.org/10.1016/j.bcmd.2004.07.006