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Title: Rescued mice with Hb E transgene-developed red cell changes similar to human β-thalassemia/HbE disease
Authors: Bundit Wannasuphaphol
Ruchaneekorn Kalpravidh
Kovit Pattanapanyasat
Panos Ioannau
Frans A. Kuypers
Suthat Fucharoen
Pranee Winichagoon
The Institute of Science and Technology for Research and Development, Mahidol University
Mahidol University
Murdoch Children's Research Institute
Children's Hospital Oakland Research Institute
Keywords: Arts and Humanities;Biochemistry, Genetics and Molecular Biology;Neuroscience
Issue Date: 1-Jan-2005
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.
ISSN: 00778923
Appears in Collections:Scopus 2001-2005

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