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Impact of α-Globin Gene Expression and α-Globin Modifiers on the Phenotype of β-Thalassemia and Other Hemoglobinopathies: Implications for Patient Management.
- Source :
-
International Journal of Molecular Sciences . Mar2024, Vol. 25 Issue 6, p3400. 18p. - Publication Year :
- 2024
-
Abstract
- In this short review, we presented and discussed studies on the expression of globin genes in β-thalassemia, focusing on the impact of α-globin gene expression and α-globin modifiers on the phenotype and clinical severity of β-thalassemia. We first discussed the impact of the excess of free α-globin on the phenotype of β-thalassemia. We then reviewed studies focusing on the expression of α-globin-stabilizing protein (AHSP), as a potential strategy of counteracting the effects of the excess of free α-globin on erythroid cells. Alternative processes controlling α-globin excess were also considered, including the activation of autophagy by β-thalassemia erythroid cells. Altogether, the studies reviewed herein are expected to have a potential impact on the management of patients with β-thalassemia and other hemoglobinopathies for which reduction in α-globin excess is clinically beneficial. [ABSTRACT FROM AUTHOR]
- Subjects :
- *GENE expression
*GLOBIN genes
*PHENOTYPES
*SICKLE cell anemia
Subjects
Details
- Language :
- English
- ISSN :
- 16616596
- Volume :
- 25
- Issue :
- 6
- Database :
- Academic Search Index
- Journal :
- International Journal of Molecular Sciences
- Publication Type :
- Academic Journal
- Accession number :
- 176333151
- Full Text :
- https://doi.org/10.3390/ijms25063400