| Autor: |
Maria Francesca Manchinu, Michela Simbula, Cristian Antonio Caria, Ester Musu, Lucia Perseu, Susanna Porcu, Maristella Steri, Daniela Poddie, Jessica Frau, Eleonora Cocco, Laura Manunza, Susanna Barella, Maria Serafina Ristaldi |
| Jazyk: |
angličtina |
| Rok vydání: |
2020 |
| Předmět: |
|
| Zdroj: |
Frontiers in Medicine, Vol 7 (2020) |
| Druh dokumentu: |
article |
| ISSN: |
2296-858X |
| DOI: |
10.3389/fmed.2020.00163 |
| Popis: |
Beta hemoglobinopathies are widely spread monogenic lethal diseases. Delta-globin gene activation has been proposed as a possible approach for curing these pathologies. The therapeutic potential of delta-globin, the non-alpha component of Hemoglobin A2 (α2δ2; HbA2), has been demonstrated in a mouse model of beta thalassemia, while its anti-sickling effect, comparable to that of gamma globin, was established some time ago. Here we show that the delta-globin mRNA level is considerably increased in a Deoxyribonuclease II-alpha knockout mouse model in which type 1 interferon (interferon beta, IFNb) is activated. IFNb activation in the fetal liver improves the delta-globin mRNA level, while the beta-globin mRNA level is significantly reduced. In addition, we show that HbA2 is significantly increased in patients with multiple sclerosis under type 1 interferon treatment. Our results represent a proof of principle that delta-globin expression can be enhanced through the use of molecules. This observation is potentially interesting in view of a pharmacological approach able to increase the HbA2 level. |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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