What is the cause of sickling of RBC in sickle cell disease?
High-Yield Explanation
In sickle cell disease glutamic acid is replaced by valine. The charge and location of this substitution cause HbS to be conveed from a soluble state into a polymer when it undergoes structural changes that accompany release of oxygen. Hypoxia, acidosis, and hypeonicity facilitate polymer formation. The polymerization of hemoglobin cause the red cell to transform from a deformable, biconcave disk into a rigid, sickle shaped cell. Ref: Rudolph's Fundamentals of Pediatrics By Abraham M. Rudolph, Pages 528-30