Affiliation:
1. From the Departments of Medicine and of Genetics and Development, Columbia University, New York, NY.
Abstract
AbstractThe human globin genes are among the most extensively characterized in the human genome, yet the details of the molecular events regulating normal human hemoglobin switching and the potential reactivation of fetal hemoglobin in adult hematopoietic cells remain elusive. Recent discoveries demonstrate physical interactions between the β locus control region and the downstream structural γ- and β-globin genes, and with transcription factors and chromatin remodeling complexes. These interactions all play roles in globin gene expression and globin switching at the human β-globin locus. If the molecular events in hemoglobin switching were better understood and fetal hemoglobin could be more fully reactivated in adult cells, the insights obtained might lead to new approaches to the therapy of sickle cell disease and β thalassemia by identifying specific new targets for molecular therapies.
Publisher
American Society of Hematology
Subject
Cell Biology,Hematology,Immunology,Biochemistry
Reference130 articles.
1. Bank A, Mears JG, Ramirez F. Disorders of human hemoglobin. Science.1980;207: 486-493.
2. Stamatoyannopoulos G, Grosveld F. Hemoglobin switching. In: Stamatoyannopoulos G, Majerus PW, Perlmutter RM, Varmus H, eds. The Molecular Basis of Blood Disease. 3rd ed. Philadelphia, PA: Saunders; 2001: 135-183.
3. Tuan D, Solomon W, Li O, London IM. The “β-like-globin” gene domain in human erythroid cells. Proc Natl Acad Sci U S A.1985;82: 6384-6388.
4. Grosveld F, van Assendelft GB, Greaves DR, Kollias G. Position-independent, high-level expression of the human β-globin gene in transgenic mice. Cell.1987;51: 975-985.
5. Tuan DYH, Solomon WB, London IM, Lee DP. An erythroid-specific, developmental-stage-independent enhancer far upstream of the human “β-like globin” genes. Proc Natl Acad Sci U S A.1989;86: 2554-2558.
Cited by
178 articles.
订阅此论文施引文献
订阅此论文施引文献，注册后可以免费订阅5篇论文的施引文献，订阅后可以查看论文全部施引文献