4. (10 pts) Hemoglobin S or sickle cell hemoglobin forms fibers in red blood cells because...

4. (10 pts) Hemoglobin S or sickle cell hemoglobin forms fibers in red blood cells because
of a Glu→Val mutation on the surface of the protein. The mutated residue interacts
with Leu and Phe residues located on the surface of a different protein molecule. This
interaction drives the formation of the fibers. Explain why higher concentrations are needed to form fibers at lower temperatures.

Expert Solution

Ans. In sickle cell hemoglobin the changed or mutated amino acid is hydrophobic (Valine amino acid in place of glutamic acide in normal hemoglobine). Hydrophobic means 'fear of water. Thus valine tends to be away from water. This chane favor the mutated hemoblobin (HbS) to form fibes to remail away from water.

Fiber formation only occurs in the deoxy or T-state / when hemoglobin (HbS) is not bound with oxygen. Higher temperatures lead to faster polymerization of HbS and increases supersaturation of HbS , lower temperature leads to slower nucleation of HbS hemoblobin and growth of fiber since it reduce the supersatuartion / or increase the solubility of HbS.

Thus sickle‐cell haemoglobin (HbS) polymers / fibers are formed in supersaturated HbS solutionto. It means to increse HbS fiber formation, supersaturaion can be achieved by increasing the concentration of HbS even at low temperature. It will increase nucleation of HbS.

gladiator answered 1 year ago

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