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|Title:||Rescued mice with Hb E transgene-developed red cell changes similar to human β-thalassemia/HbE disease|
Frans A. Kuypers
The Institute of Science and Technology for Research and Development, Mahidol University
Murdoch Children's Research Institute
Children's Hospital Oakland Research Institute
|Keywords:||Arts and Humanities;Biochemistry, Genetics and Molecular Biology;Neuroscience|
|Citation:||Annals of the New York Academy of Sciences. Vol.1054, (2005), 407-416|
|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.|
|Appears in Collections:||Scopus 2001-2005|
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