MadSci Network: Cell Biology

Re: Can we stop hydrophobic spots from sticking to each other?

Date: Tue Jun 8 17:22:26 2004
Posted By: Richard Deem, Staff, Inflammatory Bowel Disease, Cedars-Sinai Medical Center
Area of science: Cell Biology
ID: 1084475449.Cb

Sickle cell anemia is caused by a genetic mutation in the beta globin 
gene of hemoglobin. The most common mutation involves the substitution of 
the hydrophobic amino acid valine in place of hydrophilic glutamic acid 
at the sixth amino acid position of the protein. This substitution 
creates a hydrophobic spot on the outside of the protein structure that 
sticks to the hydrophobic region of an adjacent hemoglobin molecule's 
beta chain. This clumping together of the molecules into rigid fibers 
causes the "sickling" of red blood cells.

There is no way to prevent the mutant hemoglobin from sticking together, 
since this is a chemical reaction. However, it could be possible to 
genetically engineer the hematopoetic stem cells to produce the non-
mutant form of hemoglobin. This procedure would involve some sort of gene 
transfer followed by a transplant of the engineered cells back into the 
patient. In order to be effective, the old hematopoetic stem cells would 
have to be destroyed first, which would be a risky procedure, if the 
transplant did not take. In fact, a bone marrow transplant from a healthy 
patient would cure sickle cell anemia. However, the risks are deemed too 
severe to make this a standard medical procedure.

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