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.