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 | |
| State | Published - Nov 2004 |
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Keywords
- Hemoglobin E
- Homozygote
- Transgenic
ASJC Scopus subject areas
- Molecular Biology
- Molecular Medicine
- Hematology
Cite this
Generation of transgenic mice expressing human hemoglobin E. / Chen, Qiuying; Bouhassira, Eric E.; Besse, Arnaud; Suzuka, Sandra M.; Fabry, Mary E.; Nagel, Ronald L.; Hirsch, Rhoda Elison.
In: Blood Cells, Molecules, and Diseases, Vol. 33, No. 3, 11.2004, p. 303-307.Research output: Contribution to journal › Article
}
TY - JOUR
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
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.
KW - Hemoglobin E
KW - Homozygote
KW - Transgenic
UR - http://www.scopus.com/inward/record.url?scp=7444224094&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=7444224094&partnerID=8YFLogxK
U2 - 10.1016/j.bcmd.2004.07.006
DO - 10.1016/j.bcmd.2004.07.006
M3 - Article
C2 - 15528149
AN - SCOPUS:7444224094
VL - 33
SP - 303
EP - 307
JO - Blood Cells, Molecules, and Diseases
JF - Blood Cells, Molecules, and Diseases
SN - 1079-9796
IS - 3
ER -