Bundit WannasuphapholRuchaneekorn KalpravidhKovit PattanapanyasatPanos IoannauFrans A. KuypersSuthat FucharoenPranee WinichagoonThe Institute of Science and Technology for Research and Development, Mahidol UniversityMahidol UniversityMurdoch Children's Research InstituteChildren's Hospital Oakland Research Institute2018-06-212018-06-212005-01-01Annals of the New York Academy of Sciences. Vol.1054, (2005), 407-416007789232-s2.0-29744467166https://repository.li.mahidol.ac.th/handle/20.500.14594/16252A 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.Mahidol UniversityArts and HumanitiesBiochemistry, Genetics and Molecular BiologyNeuroscienceConference PaperSCOPUS10.1196/annals.1345.049