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Posted: March 24th, 2024
Cystic Fibrosis and CFTR
Organelles can contribute or cause a disease like Cystic Fibrosis. First the organelle itself may be defective because its molecules do not function well or because there has been damage to it by exposure to some harmful substance such as a chemical. Within the endoplasmic reticulum or ER where the synthesis of this protein occurs there may be a disturbance in the functions. Normally the proteins are coded within the ER for normal production and functioning of CFTR. It he protein is misfolded during the processing a disease like Cystic Fibrosis may occur.
The belief is that the PH of the CFTR protein is altered and because of this PH changes the surface tension of the CFTR changes. That change in surface tension changes the trafficking of the protein and mucus causing thicker mucus to get trapped in several organs but mostly in the lungs and pancreas. Because the surface tension is changed it becomes nearly impossible for the patient to move the mucus there for it accumulates in the lungs and is very sticky. Bacteria more easily bind to the sticky protein causing consistent infections in the lungs.
The missing or defective membrane proteins that are causing the CF become the reason why there are so many increased secretions but the worst of the problem is that with the change in the PH, the consistency of the secretions have changed and the person with the CF can just not handle them. That with the increased infections and the fact that this continues to happen throughout the other organs causes the patient to be extremely debilitated with the chance of early loss of life.
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