Fundamentals of Medicinal Chemistry

(Brent) #1

Appendix


A.1 Sickle-cell anaemia


Sickle-cell anaemia is caused by a defective gene. This gene results in the

replacement of the glutamate at position 6 of thebchains of haemoglobin

(Hb) by a valine residue to produce sickle-cell haemoglobin (HbS). These HbS

molecules aggregate into long polymer-like structures, which results in long

sickle shaped red blood cells instead of the normal round cells. These red

blood cells are able to transport oxygen in the normal fashion but their shape

causes them to lodge in capillaries, which causes tissue damage and impairs

blood circulation. This results in headaches, dizziness and ultimately death.

Fundamentals of Medicinal Chemistry, Edited by Gareth Thomas
#2003 John Wiley & Sons, Ltd
ISBN 0 470 84306 3 (Hbk), ISBN 0 470 84307 1 (pbk)

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