What is the basic hemoglobin defect in the thalassemias?
a. One of the globin chains has an amino acid substitution.
b. A structurally normal globin chain is absent or produced at lower levels.
c. Heme is produced at a lower concentration.
d. Iron is not incorporated into the protoporphyrin ring to form heme.
ANS: B
The thalassemias are a varied group of disorders in which the synthesis of a normal globin chain is reduced or absent. The globin chains that are made are normal in amino acid structure.
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