Page 289 - Biochemistry PharmD General

Biochemistry of Abnormality in the Hemoglobin

HbS or Sickle Cell Hemoglobin

• Sickle cell hemoglobin (HbS) arises due to the
defect in β chain in which glutamic acid present at
the 6th position is replaced by valine.

• Valine is also present naturally at position one.

• These two valine residues form hydrophobic
interaction producing a sticky patch on HbS.

• Due to this replacement there is a sticky patch on
HbS which appears on the oxy HbS.

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