Molecular Mechanisms of Hemoglobin F Induction
Hemoglobin F (HbF, α2γ2) is a major contributor to the clinical heterogeneity and ameliorating agent observed in patients with the β-globin disorders including β-thalassemia and sickle cell disease (SCD). During fetal life, HbF is the major hemoglobin but is lar...
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Tehran University of Medical Sciences
2011-10-01
|
| Series: | International Journal of Hematology-Oncology and Stem Cell Research |
| Subjects: | |
| Online Access: | http://journals.tums.ac.ir/PdfMed.aspx?pdf_med=/upload_files/pdf/20851.pdf&manuscript_id=20851 |
| _version_ | 1797708070171705344 |
|---|---|
| author | Najmaldin Saki Ali Dehghani Fard Majid Farshdousti Hagh Mohammad Shahjahani Saied Kaviani |
| author_facet | Najmaldin Saki Ali Dehghani Fard Majid Farshdousti Hagh Mohammad Shahjahani Saied Kaviani |
| author_sort | Najmaldin Saki |
| collection | DOAJ |
| description | Hemoglobin F (HbF, α2γ2) is a major contributor to the clinical heterogeneity and ameliorating agent observed in patients with the β-globin disorders including β-thalassemia and sickle cell disease (SCD). During fetal life, HbF is the major hemoglobin but is largely substituted by adult hemoglobin (HbA, α2β2) following a globin expression switch after birth. Increased γ-globin expression can reduce the clinical severity of β-thalassemia and SCD. Therefore, increase in HbF production has served as a longstanding goal. The progression of target-based therapeutics has been confused by limited comprehension of molecular mechanisms of gamma-globin gene expression. However, recent discoveries of regulators of HbF level represent a major development and provide new opportunities in employing novel rational therapeutic strategies. In this review, molecular mechanisms of hemoglobin F induction will be discussed. |
| first_indexed | 2024-03-12T06:15:50Z |
| format | Article |
| id | doaj.art-8e694da689cc4bdda2563d4009157dd0 |
| institution | Directory Open Access Journal |
| issn | 2008-3009 2008-2207 |
| language | English |
| last_indexed | 2024-03-12T06:15:50Z |
| publishDate | 2011-10-01 |
| publisher | Tehran University of Medical Sciences |
| record_format | Article |
| series | International Journal of Hematology-Oncology and Stem Cell Research |
| spelling | doaj.art-8e694da689cc4bdda2563d4009157dd02023-09-03T02:34:11ZengTehran University of Medical SciencesInternational Journal of Hematology-Oncology and Stem Cell Research2008-30092008-22072011-10-015459Molecular Mechanisms of Hemoglobin F InductionNajmaldin SakiAli Dehghani FardMajid Farshdousti HaghMohammad ShahjahaniSaied KavianiHemoglobin F (HbF, α2γ2) is a major contributor to the clinical heterogeneity and ameliorating agent observed in patients with the β-globin disorders including β-thalassemia and sickle cell disease (SCD). During fetal life, HbF is the major hemoglobin but is largely substituted by adult hemoglobin (HbA, α2β2) following a globin expression switch after birth. Increased γ-globin expression can reduce the clinical severity of β-thalassemia and SCD. Therefore, increase in HbF production has served as a longstanding goal. The progression of target-based therapeutics has been confused by limited comprehension of molecular mechanisms of gamma-globin gene expression. However, recent discoveries of regulators of HbF level represent a major development and provide new opportunities in employing novel rational therapeutic strategies. In this review, molecular mechanisms of hemoglobin F induction will be discussed.http://journals.tums.ac.ir/PdfMed.aspx?pdf_med=/upload_files/pdf/20851.pdf&manuscript_id=20851Hemoglobin Fβ-thalassemiaExpressionInduction |
| spellingShingle | Najmaldin Saki Ali Dehghani Fard Majid Farshdousti Hagh Mohammad Shahjahani Saied Kaviani Molecular Mechanisms of Hemoglobin F Induction International Journal of Hematology-Oncology and Stem Cell Research Hemoglobin F β-thalassemia Expression Induction |
| title | Molecular Mechanisms of Hemoglobin F Induction |
| title_full | Molecular Mechanisms of Hemoglobin F Induction |
| title_fullStr | Molecular Mechanisms of Hemoglobin F Induction |
| title_full_unstemmed | Molecular Mechanisms of Hemoglobin F Induction |
| title_short | Molecular Mechanisms of Hemoglobin F Induction |
| title_sort | molecular mechanisms of hemoglobin f induction |
| topic | Hemoglobin F β-thalassemia Expression Induction |
| url | http://journals.tums.ac.ir/PdfMed.aspx?pdf_med=/upload_files/pdf/20851.pdf&manuscript_id=20851 |
| work_keys_str_mv | AT najmaldinsaki molecularmechanismsofhemoglobinfinduction AT alidehghanifard molecularmechanismsofhemoglobinfinduction AT majidfarshdoustihagh molecularmechanismsofhemoglobinfinduction AT mohammadshahjahani molecularmechanismsofhemoglobinfinduction AT saiedkaviani molecularmechanismsofhemoglobinfinduction |