The Molecular and Cellular basis of Cystic Fibrosis

Cystic Fibrosis and CFTR

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...