But thalassemia is different: Instead
of abnormal hemoglobin, the main problem is a decreased amount of normal globin.
In 1948 British biochemist and evolutionary biologist J. B. S. Haldane noticed that the distribution of populations
with abnormal hemoglobin coincides with the distribution of malaria.
A single DNA mutation causes the body to make sticky, crescent - shaped red blood cells that
contain abnormal hemoglobin and can block blood flow in limbs and organs.
Good genes can help In 1948 British biochemist and evolutionary biologist J. B. S. Haldane noticed that the distribution of populations with
abnormal hemoglobin coincides with the distribution of malaria.
Did genes
for abnormal hemoglobin survive and spread, he wondered, because they protected against malaria infection?
Sickle cell disease is a group of inherited blood disorders caused by genetic mutations in the HBB gene, resulting
in abnormal hemoglobin, the iron - containing protein that normally allows blood cells to carry oxygen.
People with hemoglobin H disease have only moderate anemia, but they produce
an abnormal hemoglobin called hemoglobin H containing of four b chains.
Abnormal hemoglobin, the protein that enables red blood cells to carry oxygen, causes sickle cells to acquire their crescent shape and rigidity.
Due to the breakdown of
this abnormal hemoglobin, the spleen enlarges and may have to be removed.
It arises when a person inherits two copies of
an abnormal hemoglobin gene — one from each parent.
In essence, Buchanan explained, the therapy «converted» the patient to sickle - cell trait — that is, a person who carries only one copy of
the abnormal hemoglobin gene.