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Division of Genetics

Hemoglobin

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.

*Hemoglobin in 3D. This is an excellent site that displays a rotating hemoglobin molecule in 3D. (You will need chime software to view it which you can download from the site.)

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 alpha symbol, beta beta symbol, gamma gamma symbol, delta delta symbol, epsilon epsilon symbol, and zeta zeta symbol.

Normal adult hemoglobin (HbA) contains 2 alpha globin chains and 2 beta globin chains.

Fetal hemoglobin (HbF) contains 2 alpha and 2 gamma globin chains.

The embryonic hemoglobins, Gower 1 (zeta sub-2epsilon sub-2), Gower 2 (alpha sub-2epsilon sub-2) and Portland (zeta sub-2gamma sub-2), are synthesized in the very early embryo. At about 8 weeks of gestation the fetal liver takes over synthesizing predominantly HbF (alpha sub-2gamma sub-2) and a small amount of HbA (alpha sub-2beta sub-2).

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 alpha sub-2beta sub-2 >96%
HbF alpha sub-2gamma sub-2 <1%
HbA2 alpha sub-2delta sub-2 <3.5%

Elevated HbA2 is characteristic of beta-thalassemia trait.

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