Model structure of HbS showing the sites of mutations in the β subunit. The ribbon drawing was generated from the coordinates for the crystal structure of the CO form of HbS (PDB code 5E6E). The side-chain orientations for Val-6, His-63, His-92, and Cys-93 were taken from this structure, and bound CO is shown attached to the iron atom (orange sphere). The side-chain orientation of Tyr-41 was generated by adding the OH group to Phe-41 in the original structure. The orientation of the Asp-82 was taken from the structure of rHb0.1Prov-CO (PDB code 5SW7). Distance from the heme to Tyr-41 is 10.4 Å, whereas Asp-82 is located 18.1 Å away from the heme. The structure was drawn using the PyMOL Molecular Graphics System, Version 2.0 (Schrodinger, LLC).