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describe the structure and effects of RBC’s that contain sickle cell hemoglobin molecules

describe the structure and effects of RBC’s that contain sickle cell hemoglobin molecules
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Structure of RBC in Normal HbA and Sickle cell HbS  

In normal person, HbA consist of two alpha and two beta chains results in biconcave shape of RBC. Person with sickle cell hemoglobin (HbS) also carry same 2 alpha and 2 beta chains but difference is at 6 position where glutamate is replaced by valine amino acid residue and this is the only difference between HbA and HbS.

This results in abnormal quartnery structure of Hb and makes deoxyHbS less soluble than deoxyHbA.

These insoluble deoxyHbS clustered together to form tubular fibres which distorts the RBC structure into elongated sickle shape structure.

Effects of Sickle cell hemoglobin

RBC carrying sickle cell Hb not able to move easily and can cause blockage in blood vessels and this can leads to many severe problems like

1) Heart stroke and Acute chest syndrome.

2) Eye problems.

3) Delay in growth and development.

4) Joint, kidney and liver problems.

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