Hemoglobin C (abbreviated as
Hb C or
HbC) is an abnormal
hemoglobin with substitution of a
lysine residue for
glutamic acid residue at the 6th position of the β-globin chain. This mutated form reduces the normal plasticity of host
erythrocytes causing a
hemoglobinopathy. In those who are
heterozygous for the mutation, about 28–44% of total hemoglobin (Hb) is HbC, and no
anemia develops. In
homozygotes, nearly all Hb is in the HbC form, resulting in moderate
normocytic hemolytic anemia. Target cells, microspherocytes and HbC crystals are found in a
blood smear from a homozygous patient. Individuals heterozygous for both HbC and
Hb S (Hb SC disease) or for HbC and
thalassemia are known, and have atypical hemolytic anemias;
sickling is enhanced in Hb SC disease. Hemoglobin C is found predominantly in
West Africa and has been shown to protect against severe
malaria.
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