Fetal-haemoglobin enhancing genotype at BCL11A reduces HbA2 levels in patients with sickle cell anaemia.
| dc.contributor.author | Adeyemo, Titilope A | |
| dc.contributor.author | Ojewunmi, OO | |
| dc.contributor.author | Oyetunji, AI | |
| dc.contributor.author | Kalejaiye, OO | |
| dc.contributor.author | Menzel, S | |
| dc.date.accessioned | 2022-01-13T15:42:42Z | |
| dc.date.available | 2022-01-13T15:42:42Z | |
| dc.date.issued | 2021 | |
| dc.description.abstract | Understanding the interplay of genetic factors with haemoglobin expression and pathological processes in sickle cell disease is important for pharmacological and gene-therapeutic interventions. In our nascent study cohort of Nigerian patients, we found that three major disease-modifying factors, HbF levels, α-thalassaemia deletion and BCL11A genotype, had expected beneficial haematological effects. A key BCL11A variant, while improving HbF levels (5.7%–9.0%), also led to a small, but significant decrease in HbA2. We conclude that in general, interventions boosting HbF are likely to reduce HbA2 in patients’ erythroid cells and that such therapeutic strategies might benefit from a parallel stimulation of HbA2 through independent mechanisms. | en_US |
| dc.description.sponsorship | Central Research Committee of the University of Lagos (grant number: CRC 2014/07) to TA Adeyemo. S. Menzel and O. Ojewunmi are supported by the UK Medical Research Council (grant number: MR/T013389/1, to S. Menzel) | en_US |
| dc.identifier.citation | Adeyemo, TA, Ojewunmi, OO, Oyetunji, AI, Kalejaiye, OO, Menzel, S. Fetal-haemoglobin enhancing genotype at BCL11A reduces HbA2 levels in patients with sickle cell anaemia. eJHaem. 2021; 2: 459– 461. https://doi.org/10.1002/jha2.186 | en_US |
| dc.identifier.uri | https://ir.unilag.edu.ng/handle/123456789/10329 | |
| dc.language.iso | en | en_US |
| dc.publisher | Wiley/British Society for Haematology | en_US |
| dc.subject | Sickle cell disease | en_US |
| dc.subject | Haemoglobin A2 | en_US |
| dc.subject | BCLIIA | en_US |
| dc.title | Fetal-haemoglobin enhancing genotype at BCL11A reduces HbA2 levels in patients with sickle cell anaemia. | en_US |
| dc.type | Article | en_US |