A protein called the cystic fibrosis transmembrane conductance regulator (CFTR) is a regulated channel that facilitates passive diffusion of negatively charged chloride ions across the cell membrane. CFTR is critical for maintaining the balance of salt and water on the surface of your lung cells. People whose genomes are missing the gene encoding CFTR or encode a non-functional CFTR protein have cystic fibrosis, a disease that causes thick mucus to build up in the lungs, inhibiting lung function.
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It is activated by cellular signals (phosphorylation) and by ATP binding/hydrolysis. Show more…
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Sri K.
Cystic Fibrosis (CF) is a multisystem condition affecting many organs. Overall changes in lung physiology lead to dry airways, sticky secretions, a predisposition to chronic chest infections and bronchiectasis (scarring). The cystic fibrosis transmembrane conductance regulator (CFTR) protein helps to maintain the balance of salt and water on many surfaces in the body, such as the surface of the lung. When the protein is not working correctly, chloride -- a component of salt -- becomes trapped in cells. Without the proper movement of chloride, water cannot hydrate the cellular surface. This leads the mucus covering the cells to become thick and sticky, causing many of the symptoms associated with cystic fibrosis. What are the molecular mechanisms that could explain the causes of this disease? (One is enough)
When the CFTR protein is defective, epithelial cells can't regulate the way that chloride ions pass across cell membranes. This disrupts the balance of salt and water needed to maintain a normal thin coating of mucus inside the lungs and other passageways. The mucus becomes thick, sticky, and hard to move, and can result in infections from bacterial colonization. 1. What symptoms lead Dr. Weyland to his initial diagnosis? 2. Sweat glands cool the body by releasing sweat from the lower layers of the skin onto the surface. Why does a person with cystic fibrosis have salty tasting skin? 3. Consider what you know about TONICITY and the cell membrane. Why is it important to regulate salt in cells? CFTR is a ligand-gated channel, that when an ATP molecule binds, it causes a conformational change in the channel, leading to it's opening.
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