Hemoglobin subunit gamma-1 (HBG1) (HBG1)

Hemoglobin subunit gamma-1 (HBG1) and its paralog gamma-2 (HBG2) are the protein components of fetal hemoglobin (HbF), the primary oxygen carrier during fetal development. HbF possesses a higher affinity for oxygen than adult hemoglobin (HbA), facilitating the extraction of oxygen from maternal circulation across the placenta. Shortly after birth, a developmental switch occurs where HbF production is silenced and replaced by adult hemoglobin, a process regulated by repressors such as BCL11A. In patients with hemoglobinopathies like sickle cell disease or beta-thalassemia, reactivating the expression of these fetal hemoglobin genes can compensate for the lack of functional adult beta-globin. Modern therapeutic strategies, including CRISPR-based gene editing of the BCL11A enhancer or direct gene addition of modified gamma-globin sequences, aim to induce high levels of HbF to prevent the polymerization of sickle hemoglobin and improve erythropoiesis.

Other names

HBG1HBG2Gamma-globinHbFFetal hemoglobinHemoglobin subunit gamma-2

Induction of fetal hemoglobin expression via BCL11A inhibition or direct gene addition