Effect of Glucocorticosteroids in Diamond-Blackfan Anaemia: Maybe Not as Elusive as It Seems
Diamond-Blackfan anaemia (DBA) is a red blood cell aplasia that in the majority of cases is associated with ribosomal protein (RP) aberrations. However, the mechanism by which this disorder leads to such a specific phenotype remains unclear. Even more elusive is the reason why non-specific agents su...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-02-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | https://www.mdpi.com/1422-0067/23/3/1886 |
| _version_ | 1797487107749445632 |
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| author | Zuzana Macečková Agáta Kubíčková Juan Bautista De Sanctis Marian Hajdúch |
| author_facet | Zuzana Macečková Agáta Kubíčková Juan Bautista De Sanctis Marian Hajdúch |
| author_sort | Zuzana Macečková |
| collection | DOAJ |
| description | Diamond-Blackfan anaemia (DBA) is a red blood cell aplasia that in the majority of cases is associated with ribosomal protein (RP) aberrations. However, the mechanism by which this disorder leads to such a specific phenotype remains unclear. Even more elusive is the reason why non-specific agents such as glucocorticosteroids (GCs), also known as glucocorticoids, are an effective therapy for DBA. In this review, we (1) explore why GCs are successful in DBA treatment, (2) discuss the effect of GCs on erythropoiesis, and (3) summarise the GC impact on crucial pathways deregulated in DBA. Furthermore, we show that GCs do not regulate DBA erythropoiesis via a single mechanism but more likely via several interdependent pathways. |
| first_indexed | 2024-03-09T23:42:33Z |
| format | Article |
| id | doaj.art-14ece027ad274b7db3cc1b0fd81c47f3 |
| institution | Directory Open Access Journal |
| issn | 1661-6596 1422-0067 |
| language | English |
| last_indexed | 2024-03-09T23:42:33Z |
| publishDate | 2022-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | International Journal of Molecular Sciences |
| spelling | doaj.art-14ece027ad274b7db3cc1b0fd81c47f32023-11-23T16:48:15ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672022-02-01233188610.3390/ijms23031886Effect of Glucocorticosteroids in Diamond-Blackfan Anaemia: Maybe Not as Elusive as It SeemsZuzana Macečková0Agáta Kubíčková1Juan Bautista De Sanctis2Marian Hajdúch3Institute of Molecular and Translational Medicine, Palacky University Olomouc, Hnevotinska 1333/5, 77900 Olomouc, Czech RepublicInstitute of Molecular and Translational Medicine, Palacky University Olomouc, Hnevotinska 1333/5, 77900 Olomouc, Czech RepublicInstitute of Molecular and Translational Medicine, Palacky University Olomouc, Hnevotinska 1333/5, 77900 Olomouc, Czech RepublicInstitute of Molecular and Translational Medicine, Palacky University Olomouc, Hnevotinska 1333/5, 77900 Olomouc, Czech RepublicDiamond-Blackfan anaemia (DBA) is a red blood cell aplasia that in the majority of cases is associated with ribosomal protein (RP) aberrations. However, the mechanism by which this disorder leads to such a specific phenotype remains unclear. Even more elusive is the reason why non-specific agents such as glucocorticosteroids (GCs), also known as glucocorticoids, are an effective therapy for DBA. In this review, we (1) explore why GCs are successful in DBA treatment, (2) discuss the effect of GCs on erythropoiesis, and (3) summarise the GC impact on crucial pathways deregulated in DBA. Furthermore, we show that GCs do not regulate DBA erythropoiesis via a single mechanism but more likely via several interdependent pathways.https://www.mdpi.com/1422-0067/23/3/1886Diamond-Blackfan anaemiaglucocorticosteroiderythropoiesisGATA1c-mycmTOR |
| spellingShingle | Zuzana Macečková Agáta Kubíčková Juan Bautista De Sanctis Marian Hajdúch Effect of Glucocorticosteroids in Diamond-Blackfan Anaemia: Maybe Not as Elusive as It Seems International Journal of Molecular Sciences Diamond-Blackfan anaemia glucocorticosteroid erythropoiesis GATA1 c-myc mTOR |
| title | Effect of Glucocorticosteroids in Diamond-Blackfan Anaemia: Maybe Not as Elusive as It Seems |
| title_full | Effect of Glucocorticosteroids in Diamond-Blackfan Anaemia: Maybe Not as Elusive as It Seems |
| title_fullStr | Effect of Glucocorticosteroids in Diamond-Blackfan Anaemia: Maybe Not as Elusive as It Seems |
| title_full_unstemmed | Effect of Glucocorticosteroids in Diamond-Blackfan Anaemia: Maybe Not as Elusive as It Seems |
| title_short | Effect of Glucocorticosteroids in Diamond-Blackfan Anaemia: Maybe Not as Elusive as It Seems |
| title_sort | effect of glucocorticosteroids in diamond blackfan anaemia maybe not as elusive as it seems |
| topic | Diamond-Blackfan anaemia glucocorticosteroid erythropoiesis GATA1 c-myc mTOR |
| url | https://www.mdpi.com/1422-0067/23/3/1886 |
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