دورية أكاديمية
أعرض في EDS

Stem cell transplantation demonstrates that Sox6 represses epsilon y globin expression in definitive erythropoiesis of adult mice.

التفاصيل البيبلوغرافية
العنوان: Stem cell transplantation demonstrates that Sox6 represses epsilon y globin expression in definitive erythropoiesis of adult mice.
المؤلفون: Cohen-Barak O; Department of Pediatrics, University of Arizona, College of Medicine, Tucson, AZ 85724, USA., Erickson DT, Badowski MS, Fuchs DA, Klassen CL, Harris DT, Brilliant MH
المصدر: Experimental hematology [Exp Hematol] 2007 Mar; Vol. 35 (3), pp. 358-67.
نوع المنشور: Journal Article; Research Support, N.I.H., Extramural
اللغة: English
بيانات الدورية: Publisher: Elsevier Science Inc Country of Publication: Netherlands NLM ID: 0402313 Publication Model: Print Cited Medium: Print ISSN: 0301-472X (Print) Linking ISSN: 0301472X NLM ISO Abbreviation: Exp Hematol Subsets: MEDLINE
أسماء مطبوعة: Publication: 1999- : Amsterdam : Elsevier Science Inc.
Original Publication: Copenhagen, Munksgaard.
مواضيع طبية MeSH: Stem Cell Transplantation*, DNA-Binding Proteins/*immunology , Erythropoiesis/*genetics , Gene Expression Regulation/*genetics , Globins/*genetics , High Mobility Group Proteins/*immunology , Transcription Factors/*immunology, Animals ; DNA-Binding Proteins/deficiency ; Erythroid Cells/immunology ; Female ; Globins/biosynthesis ; Graft Survival ; High Mobility Group Proteins/deficiency ; Liver/cytology ; Mice ; Mice, Transgenic ; Pregnancy ; Promoter Regions, Genetic/genetics ; RNA, Messenger/genetics ; SOXD Transcription Factors ; Transcription Factors/deficiency ; Transcription, Genetic ; Transfection ; Transplantation Conditioning ; Transplantation, Homologous
مستخلص: Sox6, a member of the Sox transcription factor family, is essential for the silencing of epsilon y globin gene expression in definitive erythropoiesis of mice. Homozygous Sox6-null mice are neonatally lethal, precluding analysis at later stages. We created adult mice that are deficient in Sox6 specifically in hematopoietic tissues by transplanting embryonic liver stem cells from Sox6-deficient mice into lethally irradiated congenic wild-type adult mice. The mice receiving mutant stem cells (mutant engrafted) showed high expression levels of epsilon y in bone marrow, spleen, and circulating blood compared with mice receiving wild-type and heterozygous stem cells (control engrafted). The level of expression of epsilon y in circulating blood was directly correlated with the percentage of successful mutant donor cell engraftment. Additionally, the mutant engrafted adult mice showed an increase in erythroid precursor cells in bone marrow, spleen, and blood. Thus, Sox6 continues to function as a major regulator of epsilon y in adult definitive erythropoiesis and is required for normal erythrocyte maturation. Therefore, Sox6 may provide a novel therapeutic target by reactivating epsilon y in patients with hemoglobinopathies such as sickle cell anemia and beta-thalassemia.
معلومات مُعتمدة: CA23074 United States CA NCI NIH HHS
المشرفين على المادة: 0 (DNA-Binding Proteins)
0 (High Mobility Group Proteins)
0 (RNA, Messenger)
0 (SOXD Transcription Factors)
0 (Sox6 protein, mouse)
0 (Transcription Factors)
9004-22-2 (Globins)
تواريخ الأحداث: Date Created: 20070221 Date Completed: 20071212 Latest Revision: 20081121
رمز التحديث: 20221213
DOI: 10.1016/j.exphem.2006.11.009
PMID: 17309816
قاعدة البيانات: MEDLINE
الوصف
تدمد:0301-472X
DOI:10.1016/j.exphem.2006.11.009