First Author | Gurumurthy A | Year | 2020 |
Journal | Front Physiol | Volume | 11 |
Pages | 590180 | PubMed ID | 33101065 |
Mgi Jnum | J:329638 | Mgi Id | MGI:6730838 |
Doi | 10.3389/fphys.2020.590180 | Citation | Gurumurthy A, et al. (2020) TFII-I/Gtf2i and Erythro-Megakaryopoiesis. Front Physiol 11:590180 |
abstractText | TFII-I is a ubiquitously expressed transcription factor that positively or negatively regulates gene expression. TFII-I has been implicated in neuronal and immunologic diseases as well as in thymic epithelial cancer. Williams-Beuren Syndrome (WBS) is caused by a large hemizygous deletion on chromosome 7q11.23 which encompasses 26-28 genes, including GTF2I, the human gene encoding TFII-I. A subset of WBS patients has recently been shown to present with macrocytosis, a mild anemia characterized by enlarged erythrocytes. We conditionally deleted the TFII-I/Gtf2i gene in adult mice by tamoxifen induced Cre-recombination. Bone marrow cells revealed defects in erythro-megakaryopoiesis and an increase in expression of the adult beta-globin gene. The data show that TFII-I acts as a repressor of beta-globin gene transcription and that it is implicated in the differentiation of erythro-megakaryocytic cells. |