Cooleys anemia is...: Aka beta thalassemia major. It stems from the inheritance in an offspring of two abnormal copies of the beta globin gene. Beta globins (2 of them) partner with two Alpha globins, each with a heme prup, to form hemoglobin, the oxygen carrying pigment protein in hemoglobin. If you are deficient in beta globin, you accumulate Alpha globin that can damage red cells. Pts need frequent transfusions.
Answered 6/24/2014
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