| First Author | Chang J | Year | 1996 |
| Journal | Blood | Volume | 88 |
| Issue | 5 | Pages | 1846-51 |
| PubMed ID | 8781443 | Mgi Jnum | J:35018 |
| Mgi Id | MGI:82470 | Doi | 10.1182/blood.v88.5.1846.1846 |
| Citation | Chang J, et al. (1996) Inactivation of mouse alpha-globin gene by homologous recombination: mouse model of hemoglobin H disease. Blood 88(5):1846-51 |
| abstractText | We have disrupted the 5' locus of the duplicated adult alpha-globin genes by gene targeting in the mouse embryonic stem cells and created mice with alpha- thalassemia syndromes. The heterozygous knockout mice (*alpha/alpha alpha) are asymptomatic like the silent carriers in humans whereas the homozygous knockout mice (*alpha/*alpha) show hemolytic anemia, Mice with three dysfunctional alpha-globin genes generated by breeding the 5' alpha-globin knockouts (*alpha/alpha alpha) and the deletion type alpha-thalassemia mice (--/ alpha alpha) produce severe hemoglobin H disease and they die in utero. These results indicate that the 5' alpha-globin gene is the predominant locus in mice, and suggest that it is even more dominant than its human homologue. (C) 1996 by The American Society of Hematology. |
