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

doi:10.1016/j.bcmd.2004.07.006

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 journal › Article › peer-review

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 language	English (US)
Pages (from-to)	303-307
Number of pages	5
Journal	Blood Cells, Molecules, and Diseases
Volume	33
Issue number	3
DOIs	https://doi.org/10.1016/j.bcmd.2004.07.006
State	Published - Nov 2004

Keywords

Hemoglobin E
Homozygote
Transgenic

ASJC Scopus subject areas

Molecular Medicine
Molecular Biology
Hematology
Cell Biology

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@article{12565f151cba452b8f881d53940c3d84,

title = "Generation of transgenic mice expressing human hemoglobin E",

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.",

keywords = "Hemoglobin E, Homozygote, Transgenic",

author = "Qiuying Chen and Bouhassira, {Eric E.} and Arnaud Besse and Suzuka, {Sandra M.} and Fabry, {Mary E.} and Nagel, {Ronald L.} and Hirsch, {Rhoda Elison}",

note = "Funding Information: We thank Dr. Christine Lawrence, M.D., Distinguished University Professor of Medicine, Emeritus (Albert Einstein College of Medicine), for the red blood cell images and participation in the interpretations. This work was supported in part by the NIH R21 DK 06423, NIH Bronx Comprehensive Sickle Cell Center U54 HL 070994; NIH R01 DK 56845; P01 HL 55435; R21 DK 061799; M01 RR 12248; and the American Heart Association, Grant in Aid No. 0256390T.",

year = "2004",

month = nov,

doi = "10.1016/j.bcmd.2004.07.006",

language = "English (US)",

volume = "33",

pages = "303--307",

journal = "Blood Cells, Molecules, and Diseases",

issn = "1079-9796",

publisher = "Academic Press Inc.",

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T1 - Generation of transgenic mice expressing human hemoglobin E

AU - Chen, Qiuying

AU - Bouhassira, Eric E.

AU - Besse, Arnaud

AU - Suzuka, Sandra M.

AU - Fabry, Mary E.

AU - Nagel, Ronald L.

AU - Hirsch, Rhoda Elison

N1 - Funding Information: We thank Dr. Christine Lawrence, M.D., Distinguished University Professor of Medicine, Emeritus (Albert Einstein College of Medicine), for the red blood cell images and participation in the interpretations. This work was supported in part by the NIH R21 DK 06423, NIH Bronx Comprehensive Sickle Cell Center U54 HL 070994; NIH R01 DK 56845; P01 HL 55435; R21 DK 061799; M01 RR 12248; and the American Heart Association, Grant in Aid No. 0256390T.

PY - 2004/11

Y1 - 2004/11

N2 - 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.

AB - 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.

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