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Sickle Cell Anemia. Sickle Cell anemia is an inherited red blood cell disorder. Normal red blood cells are round like doughnuts, and they move through small blood tubes in the body to deliver oxygen.

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Sickle cell anemia

Sickle Cell Anemia

Sickle Cell anemia is an inherited red blood cell disorder. Normal red blood cells are round like doughnuts, and they move through small blood tubes in the body to deliver oxygen.

Sickle red blood cells become hard, sticky and shaped like sickles used to cut wheat. When these hard and pointed red cells go through the small blood tube, they clog the flow and break apart. This can cause pain, damage and a low blood count, or anemia.


The origin of the disease is a small change in the protein hemoglobin

The origin of the disease is a small change in the protein hemoglobin

The change in cell structure arises from a change in

the structure of hemoglobin.

A single change in an amino acid causes hemoglobin

to aggregate.


The function of hemoglobin is to carry oxygen

The function of hemoglobin is to carry oxygen hemoglobin

Hemoglobin A

Normal hemoglobin has

four subunits that each

contain an oxygen binding

site.

Biswal, B. K., Vijayan, M.:

Structures of Human Oxy-

and Deoxyhaemoglobin at

Different Levels of Humidity:

Variability in the T State

Acta Crystallogr., Sect.D

58 pp. 1155 (2002)


The origin of sickle cell anemia is a mutation in hemoglobin

The origin of sickle cell anemia is a mutation in hemoglobin hemoglobin

Hemoglobin S

A single mutation in

hemoglobin results in a

binding of one protein

to another.

Padlan, E. A., Love, W. E.: Refined crystal structure of deoxyhemoglobin S. I. Restrained least-squares refinement at 3.0-Å resolution. J Biol Chem 260 pp. 8272 (1985)


Hemoglobin is a carrier protein
Hemoglobin is a carrier protein hemoglobin

O2

HbO2

CO2

deoxy Hb (CO2)

Tissues

Lungs


Hemoglobin changes structure for efficient oxygen uptake and delivery
Hemoglobin changes structure for efficient oxygen uptake and delivery

HbO2

deoxy Hb (CO2)

Strong binding state

R state

Weak binding state

T state



The cooperative r t switch relies on iron displacement to communicate between a and b subunits
The cooperative R - T switch relies on iron displacement to communicate between a and b subunits

Hemoglobin is composed of two a and two b

subunits whose structures resemble myoglobin.

Eaton et al. Nature Struct. Biol. 1999, 6, 351


The small change in hemoglobin structure leads to aggregation

The small change in hemoglobin structure leads to aggregation

a

b

Subunits

Normal hemoglobin (Hb A)

Sickle cell hemoglobin (Hb S)


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