Porth's Essentials of Pathophysiology, 4e - page 297

278
U N I T 3
Hematopoietic Function
of a globin (protein) portion and a heme unit, which
surrounds an atom of iron that binds oxygen.
1,3
Thus,
each molecule of hemoglobin can carry four molecules
of oxygen. Hemoglobin is a natural pigment; because
of its iron content, it appears reddish when oxygen is
attached and has a bluish cast when deoxygenated. The
production of each type of globin chain is controlled by
individual structural genes with five different gene loci.
Mutations, which can occur anywhere in these five loci,
have resulted in over 550 types of abnormal hemoglobin
molecules.
1
There are two major types of normal hemoglobin—
adult hemoglobin (HbA) and fetal hemoglobin (HbF).
Adult hemoglobin consists of a pair of
α
chains and a pair
of
β
chains. Fetal hemoglobin is the predominant hemo-
globin in the fetus fromthe third through theninthmonths
of gestation. It has a pair of gamma (
γ
) chains substituted
for the
α
chains. Because of this chain substitution, HbF
has a higher affinity for oxygen than adult hemoglobin.
This affinity facilitates the transfer of oxygen across
the placenta from the HbA in the mother’s blood to
the HbF in the fetus’s blood. HbF is replaced within
6 months of birth with HbA.
FIGURE 13-1.
A highly magnified (× 11,397) electron
micrograph of a number of red blood cells found enmeshed
in a fibrinous matrix on the luminal surface of an indwelling
vascular catheter. Note the biconcave shape of each
erythrocyte, which increases the surface area of these
hemoglobin-filled cells, thus promoting more effective
gas exchange. (From the Centers for Disease Control and
Prevention Public Health Images Library. No. 7315. Courtesy of
Janice Carr.)
Hemoglobin
Hemoglobin
Heme
Red blood cell
Plasma membrane
β
2
β
1
α
2
α
1
A
B
N NFe
N
N
C
C
C
C
FIGURE 13-2.
(A)
Biconcave
structure of the red blood cell as
shown in cross-section and in
lateral surface view.
(B)
Hemoglobin
molecule, showing the four iron (Fe)-
containing heme subunits and their
structure.
Actin
Red cell
membrane
Spectrin
Red cell
antigens
Integral
protein
Ankyrin
FIGURE 13-3.
Cross-sectional side view of the biconcave
structure of the red blood cell and diagram showing the
cytoskeleton and flexible network of spectrin proteins that
attach to the ankyrin protein, a transmembrane protein that
resides on the inner surface of the membrane and is anchored
to an integral protein that spans the membrane.
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