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The Coup-TFII orphan nuclear receptor is an activator of the γ-globin gene
Università di Milano-Bicocca, Italy.
Università di Milano-Bicocca, Italy.
Università di Milano-Bicocca, Italy.
Istituto di Ricerca Genetica e Biomedica del Consiglio Nazionale delle Ricerche, Italy.
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2020 (English)In: Haematologica, ISSN 0390-6078, E-ISSN 1592-8721Article in journal (Refereed) Epub ahead of print
Abstract [en]

The human fetal γ-globin gene is repressed in the adult stage through complex regulatory mechanisms involving transcription factors and epigenetic modifiers. Reversing γ-globin repression, or maintaining its expression by manipulating regulatory mechanisms, has become a major clinical goal in the treatment of β-hemoglobinopathies. Here, we identify the orphan nuclear receptor Coup-TFII (NR2F2/ARP-1) as an embryonic/fetal stage activator of γ-globin expression. We show that Coup-TFII is expressed in early erythropoiesis of yolk sac origin, together with embryonic/fetal globins. When overexpressed in adult cells (including peripheral blood cells from human healthy donors and β039 thalassemic patients) Coup-TFII activates the embryonic/fetal globins genes, overcoming the repression imposed by the adult erythroid environment. Conversely, the knock-out of Coup-TFII increases the β/γ+β globin ratio. Molecular analysis indicates that Coup-TFII binds in vivo to the β-locus and contributes to its conformation. Overall, our data identify Coup-TFII as a specific activator of the γ-globin gene.

Place, publisher, year, edition, pages
Ferrata Storti Foundation , 2020.
Keywords [en]
Hemoglobinopathies, Red Cells, Thalassemia
National Category
Cell and Molecular Biology
Identifiers
URN: urn:nbn:se:liu:diva-164345DOI: 10.3324/haematol.2019.241224PubMedID: 32107331OAI: oai:DiVA.org:liu-164345DiVA, id: diva2:1415459
Available from: 2020-03-18 Created: 2020-03-18 Last updated: 2020-03-18

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Cantù, Claudio

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CiteExportLink to record
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