Alpha-Thalassemia
The α-Thalassemia involves the genes HBA1 and HBA2,
inherited in a Mendelian recessive fashion. Two gene loci and so four alleles
exist. It is also connected to the deletion of the 16p chromosome. α Thalassemia
result in decreased alpha-globin pro
duction, therefore fewer alpha-globin
chains are produced, resulting in an excess of β chains in adults and excess γ
chains in newborns. The excess β chains form unstable tetramers (called
hemoglobin H or HbH of 4 beta chains), which have abnormal oxygen dissociation
curves.
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