TFII-I/Gtf2i and Erythro-Megakaryopoiesis

Autor: Zhijian Qian, Rukiye Nar, John Strouboulis, Jörg Bungert, Marjorie Brand, Kimberly Paulsen, Qiong Wu, Alexis Trumbull, Aishwarya Gurumurthy, Ryan C Fishman
Rok vydání: 2020
Předmět:
Zdroj: Frontiers in Physiology, Vol 11 (2020)
Frontiers in Physiology
ISSN: 1664-042X
DOI: 10.3389/fphys.2020.590180
Popis: 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 β-globin gene. The data show that TFII-I acts as a repressor of β–globin gene transcription and that it is implicated in the differentiation of erythro-megakaryocytic cells.
Databáze: OpenAIRE