Hemoglobin is the oxygen carrying substance in the blood. It is found in the red blood cells, and it is what gives blood its red color. It is a complex molecule consisting of 4 globin chains (polypeptide chains or proteins) with a heme group inserted into each globin "pocket". The heme group consists of a porphyrin ring and an iron (Fe) atom occupying the center of the ring. It is the iron atom that binds oxygen for transport through the blood. One hemoglobinn molecule carries 4 oxygen molecules.
The normal human hemoglobins found at different stages of development are encoded for by 6 different structural genes resulting in 6 globin (polypeptide) chains: alpha , beta , gamma , delta , epsilon , and zeta .
Normal adult hemoglobin (HbA) contains 2 globin chains and 2 globin chains.
Fetal hemoglobin (HbF) contains 2 and 2 globin chains.
The embryonic hemoglobins, Gower 1 ( ), Gower 2 ( ) and Portland ( ), are synthesized in the very early embryo. At about 8 weeks of gestation the fetal liver takes over synthesizing predominantly HbF ( ) and a small amount of HbA ( ).
An infant is born with approximately 70% HbF. The level normally falls to about 1% within the first year of life.
A normal adult will typically have:
| HbA |
  |
>96% |
| HbF |
  |
<1% |
| HbA2 |
  |
<3.5% |
Elevated HbA2 is characteristic of beta-thalassemia trait.
|