What I know.... I know the only difference between sickle-cell hemoglobin andnor

Question

What I know.... I know the only difference between sickle-cell hemoglobin andnormal hemoglobin is a single amino acid substitution, valine(Val)for glutamic acid (Glu). So the structural difference is with the"R goup" since the Amino group and Carboxl group are the same inboth Valine and Glutamic. Question I have to explain why this particular amino acid substitutionhas dramtic effects on the structure and function of hemoglobin. Ido not have to explain what the effects are, but why thisparticular substitution results in these effects. What I know.... I know the only difference between sickle-cell hemoglobin andnormal hemoglobin is a single amino acid substitution, valine(Val)for glutamic acid (Glu). So the structural difference is with the"R goup" since the Amino group and Carboxl group are the same inboth Valine and Glutamic. Question I have to explain why this particular amino acid substitutionhas dramtic effects on the structure and function of hemoglobin. Ido not have to explain what the effects are, but why thisparticular substitution results in these effects.

Explanation / Answer

The particular order that the amino acids which make up Hemoglobin are arranged in are crucial to its proper function.  When valine is substituted for glutamic acid, the 3-D shape of the Hemoglobin protein molecule is completely transformed.  The hydrogen and covalent bonding in the secondary, tertiary and quaternary structures of Hemoglobin are altered, resulting in red blood cells sickling.  These blood cells have reduced lifespan (20 days as opposed to 120) and they cannot bend or move in small capillaries like normal blood cells as a result of the shape change in the Hemoglobin protein.  This also causes the manifested problems and symptoms.

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