The model shows the sequence motifs and DNA-binding proteins that mediate transcriptional regulation of HBG1 and HBG2. Organization of the human β-globin locus on chromosome 11 is at the bottom, with the 5 functional genes color coded by site of expression in the yolk sac (ε), fetal liver (Gγ and Aγ), and adult bone marrow (δ and β) (not to scale). The presence of the 3′ HS site and the core HS sites within the LCR are shown as purple boxes. The 2 CCAAT boxes are shaded, and binding to them by the NF-Y complex (A, B, and C components) is indicated. BCL11A binds to the TGACC motif highlighted in orange, and TR4 (as homo- or heterodimers with other steroid hormone receptors) binds to the DR1 sequence, which is underlined and highlighted with red/orange text. The positions of the −117 and −114 and Δ13 hereditary persistence of fetal hemoglobin mutations are indicated. NF-YA, nuclear factor Y, component A.