Hemoglobin subunit gamma-1 (HBG1)

Hemoglobin subunit gamma-1 is one of the two gamma globin chains (the other being gamma-2, or HBG2), encoded by the HBG1 gene on chromosome 11. It is primarily expressed during fetal development in the liver, spleen, and bone marrow. Together with alpha chains, it forms fetal hemoglobin (HbF, α2γ2), which is replaced by adult hemoglobin (HbA) after birth. Unlike adult β-globin, gamma chains have greater oxygen affinity, which facilitates oxygen transfer from maternal to fetal blood. Persistence of gamma globin into adulthood (hereditary persistence of fetal hemoglobin, HPFH) or its upregulation (as in response to gene therapy or pharmacological intervention) can mitigate symptoms of beta-globin disorders such as sickle cell disease and beta-thalassemia. The protein is not a direct therapeutic target but is fundamental to clinically important biomarker and modifier gene strategies focused on increasing fetal hemoglobin for disease modification

Other names

Gamma-1-globinHb F AgammaHemoglobin gamma-1 chainHemoglobin gamma-A chainHBG-T2HBGAHBGRHSGGL1PRO2979

Induction of fetal hemoglobin (HbF) by stimulating HBG1 gene expression as a strategy to ameliorate symptoms in sickle cell disease and beta-thalassemia. Mechanisms include demethylation of promoters or modulation of transcription factors to maintain or reactivate gamma globin gene expression in adults