What is the cause of sickling of RBC in sickle cell disease?
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
Decreased Solubility
Decreased Stability
Altered Function
Altered 02 binding capacity