The Roles of Mitophagy and Autophagy in Ineffective Erythropoiesis in β-Thalassemia
β-Thalassemia is one of the most common genetically inherited disorders worldwide, and it is characterized by defective β-globin chain synthesis leading to reduced or absent β-globin chains. The excess α-globin chains are the key factor leading to the death of differentiating erythroblasts in a proc...
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MDPI AG
2022-09-01
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| Series: | International Journal of Molecular Sciences |
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| author | Pornthip Chaichompoo Saovaros Svasti Duncan R. Smith |
| author_facet | Pornthip Chaichompoo Saovaros Svasti Duncan R. Smith |
| author_sort | Pornthip Chaichompoo |
| collection | DOAJ |
| description | β-Thalassemia is one of the most common genetically inherited disorders worldwide, and it is characterized by defective β-globin chain synthesis leading to reduced or absent β-globin chains. The excess α-globin chains are the key factor leading to the death of differentiating erythroblasts in a process termed ineffective erythropoiesis, leading to anemia and associated complications in patients. The mechanism of ineffective erythropoiesis in β-thalassemia is complex and not fully understood. Autophagy is primarily known as a cell recycling mechanism in which old or dysfunctional proteins and organelles are digested to allow recycling of constituent elements. In late stage, erythropoiesis autophagy is involved in the removal of mitochondria as part of terminal differentiation. Several studies have shown that autophagy is increased in earlier erythropoiesis in β-thalassemia erythroblasts, as compared to normal erythroblasts. This review summarizes what is known about the role of autophagy in β-thalassemia erythropoiesis and shows that modulation of autophagy and its interplay with apoptosis may provide a new therapeutic route in the treatment of β-thalassemia. Literature was searched and relevant articles were collected from databases, including PubMed, Scopus, Prospero, Clinicaltrials.gov, Google Scholar, and the Google search engine. Search terms included: β-thalassemia, ineffective erythropoiesis, autophagy, novel treatment, and drugs during the initial search. Relevant titles and abstracts were screened to choose relevant articles. Further, selected full-text articles were retrieved, and then, relevant cross-references were scanned to collect further information for the present review. |
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| issn | 1661-6596 1422-0067 |
| language | English |
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| spelling | doaj.art-d9e09ce81db347feb11ea987c4f060f52023-11-23T16:48:42ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672022-09-0123181081110.3390/ijms231810811The Roles of Mitophagy and Autophagy in Ineffective Erythropoiesis in β-ThalassemiaPornthip Chaichompoo0Saovaros Svasti1Duncan R. Smith2Department of Pathobiology, Faculty of Science, Mahidol University, Bangkok 10400, ThailandThalassemia Research Center, Institute of Molecular Biosciences, Mahidol University, Nakhon Pathom 73170, ThailandPathology Laboratory, Institute of Molecular Biosciences, Mahidol University, Nakhon Pathom 73170, Thailandβ-Thalassemia is one of the most common genetically inherited disorders worldwide, and it is characterized by defective β-globin chain synthesis leading to reduced or absent β-globin chains. The excess α-globin chains are the key factor leading to the death of differentiating erythroblasts in a process termed ineffective erythropoiesis, leading to anemia and associated complications in patients. The mechanism of ineffective erythropoiesis in β-thalassemia is complex and not fully understood. Autophagy is primarily known as a cell recycling mechanism in which old or dysfunctional proteins and organelles are digested to allow recycling of constituent elements. In late stage, erythropoiesis autophagy is involved in the removal of mitochondria as part of terminal differentiation. Several studies have shown that autophagy is increased in earlier erythropoiesis in β-thalassemia erythroblasts, as compared to normal erythroblasts. This review summarizes what is known about the role of autophagy in β-thalassemia erythropoiesis and shows that modulation of autophagy and its interplay with apoptosis may provide a new therapeutic route in the treatment of β-thalassemia. Literature was searched and relevant articles were collected from databases, including PubMed, Scopus, Prospero, Clinicaltrials.gov, Google Scholar, and the Google search engine. Search terms included: β-thalassemia, ineffective erythropoiesis, autophagy, novel treatment, and drugs during the initial search. Relevant titles and abstracts were screened to choose relevant articles. Further, selected full-text articles were retrieved, and then, relevant cross-references were scanned to collect further information for the present review.https://www.mdpi.com/1422-0067/23/18/10811autophagyapoptosisER stressβ-thalassemiaineffective erythropoiesis |
| spellingShingle | Pornthip Chaichompoo Saovaros Svasti Duncan R. Smith The Roles of Mitophagy and Autophagy in Ineffective Erythropoiesis in β-Thalassemia International Journal of Molecular Sciences autophagy apoptosis ER stress β-thalassemia ineffective erythropoiesis |
| title | The Roles of Mitophagy and Autophagy in Ineffective Erythropoiesis in β-Thalassemia |
| title_full | The Roles of Mitophagy and Autophagy in Ineffective Erythropoiesis in β-Thalassemia |
| title_fullStr | The Roles of Mitophagy and Autophagy in Ineffective Erythropoiesis in β-Thalassemia |
| title_full_unstemmed | The Roles of Mitophagy and Autophagy in Ineffective Erythropoiesis in β-Thalassemia |
| title_short | The Roles of Mitophagy and Autophagy in Ineffective Erythropoiesis in β-Thalassemia |
| title_sort | roles of mitophagy and autophagy in ineffective erythropoiesis in β thalassemia |
| topic | autophagy apoptosis ER stress β-thalassemia ineffective erythropoiesis |
| url | https://www.mdpi.com/1422-0067/23/18/10811 |
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