Question

Describe hemoglobin synthesis and be very detailed.

Answer 1

Hemoglobin is the essential protein which mainly found in Mature RBC's as 600 million hemoglobin molecules. It is a tetramer composed of 4 globin chains such as 2 alpha and 2 beta chains, which is derived from chromosomes 11 and 16.

Hemoglobin function as oxygen carrier of RBC's as it carries oxygen frrom lungs to tissues and returns CO2 from tissues to lungs. hemoglobin gets bind with oxygen in O2 rich environment , where it is left if oxygen is absent.

During proerythroblast stage of RBC, hemoglobin starts getting produced in ribosome and mitochondria as a result of biochemical reaction.

Heme portion of hemoglobin starts synthesizing at mitochondria with the condensation of glycine& succinyl-CoA resulting in formation of δ-aminolevulinic acid (ALA).

ALA left mitochondria for the formation of porphobilinogen and undergoes multiple reactions for the formation of coproporphyrinogen. It returns to mitochondria and forms protoporphyrin.

It [ protoporphyrin] combines with iron to produce heme. Heme then leaves the mitochondria and reaches the ribosome, combines with globulin to form hemoglobin.

Any disturbance during these steps in production of hemoglobin resulting in iron defeciency anemia, Thalassemia, lead poisoning and sideroblastic anemia.