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THALASSEMIA

 Hemoglobin synthesis requires the coordinated production of heme and globin.

 Hemoglobin synthesis begins in the mitochondrion with the condensation of succinyl-


CoA, then binds with glycine to form a pyrrole molecule. In turn, four pyrroles combine
to form protoporphyrin IX it then combines with iron to form the heme molecule. Finally,
each heme molecule combines with a long polypeptide chain, a globin synthesized by
ribosomes.

 Thalassemia is a hemoglobin disease that results from the mutation/deletion of one or


more of the globin chain in hemoglobin.

 There are 2 types of thalassemia namely, alpha thalassemia and beta thalassemia, which
are further, subdivided into 4 subtypes for alpha thalassemia and 3 subtypes for beta
thalassemia respectively.
 HbA1C, which is the glycosylated form of a hemoglobin (glucose + hemoglobin) is used
in the diagnosis and management of Diabetes Mellitus patients.

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