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Potentially
lethal human disease, inherited as an autosomal recessive, caused
by a mutation in a gene coding for the beta subunit of the oxygen-transporting
protein haemoglobin. Under conditions of low oxygen tension, the altered
beta-globin molecule causes haemoglobin to aggregate forming rod-like
arrays that distort the cell membrane forcing red blood cells to become
sickle-shaped. The sickled red blood cells are damaged and rapidly
removed from the circulation causing anaemia. |
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