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Cystic Fibrosis, What is It?
Cystic Fibrosis, What is It? Cystic Fibrosis, What is It? Cystic Fibrosis is an inherited disease that affects the tissues that produce mucus secretions. These tissues can be found in the lungs, gastrointestinal tract, pan crease, and the ducts of the liver. Cystic fibrosis can also affect the sweat glands and the male reproductive system. People with the disease have abnormally thick secretions in the lungs as well as other organs. It was originally thought by researchers in the late eighties, that patients with cystic fibrosis had a faulty chloride channel, a gate in the cell membrane that regulates the entry of chloride. They proved through their research that the epithelial cells, which lined the body’s airways, contained this malfunctioning channel. The lack of a correctly working chloride gateway results in airway surface fluid that is loaded with salt, hence, one of the symptoms of cystic fibrosis, salty skin. Researchers suspected that this faulty channel lead to the heavy mucus that blocked the airways path, eventually leading to death. It wasn’t until the nineties when researchers found that cystic fibrosis is caused by mutations in a protein called CFTR (cystic fibrosis transmembrane conductance regulator), those mutations being that proteins are absence in the cell membrane. fibrosis, cystic, drug, chloride, cftr, therapy, mutations, disease, class, protein, channel, cell, mucus, membrane, called, salt, researchers, proteins, patients, one, new, lungs, given, cells, being, absence, years, ways, under, trafficking, tobi, tissues, through, thick, therapeutic, surface, secretions, respiratory, reduced, protein-repair, production, person, people, move, maintain, leading, infections, improved, iii, ii
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