Publication: Rescued mice with Hb E transgene-developed red cell changes similar to human β-thalassemia/HbE disease
Issued Date
2005-01-01
Resource Type
ISSN
00778923
Other identifier(s)
2-s2.0-29744467166
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Mahidol University
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SCOPUS
Bibliographic Citation
Annals of the New York Academy of Sciences. Vol.1054, (2005), 407-416
Suggested Citation
Bundit Wannasuphaphol, Ruchaneekorn Kalpravidh, Kovit Pattanapanyasat, Panos Ioannau, Frans A. Kuypers, Suthat Fucharoen, Pranee Winichagoon Rescued mice with Hb E transgene-developed red cell changes similar to human β-thalassemia/HbE disease. Annals of the New York Academy of Sciences. Vol.1054, (2005), 407-416. doi:10.1196/annals.1345.049 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/16252
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Title
Rescued mice with Hb E transgene-developed red cell changes similar to human β-thalassemia/HbE disease
Abstract
A novel C57BL/6 transgenic murine model of HbE has been developed, and the heterotetrameric (IIIα2hβE2) hemoglobin shows significant complementation of mild thalassemia phenotype in double heterozygous (βm+βm-, βhE) and homozygous knockout (βm-βm-, βhE) mice with 100% heterotetrameric hemoglobin. Lethal homozygous β-thalassemic mice rescued by HbE transgenes mimic β-thalassemia/HbE phenotype in human. Although anemia was not pronounced, other hematologic parameters were abnormally similar to β-knockout mice. Flow cytometric study revealed a highly oxidative status in the red cells, but there were no marked changes in PS red cells and RBC vesicles. RBC life span and half-time of rescued red cells were shortened, indicating a rapid RBC destruction. © 2005 New York Academy of Sciences.