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Cystic Fibrosis

Cystic Fibrosis is incurable hereditary disorder that causes the body to secrete an abnormally thick, sticky mucus that clogs the pancreas and the lungs , leading to problems with breathing and digestion, infection, and ultimately, death. Three decades ago most babies born with cystic fibrosis died in early childhood, but advances in diagnosing and treating the disease have significantly improved its prognosis. Cystic fibrosis is caused by a defect in the gene responsible for manufacturing cystic fibrosis transmembrane conductance regulator (CFTR), a protein that controls the flow of chloride ions into and out of certain cells. In healthy people, CFTR forms a channel in the plasma membrane through which chloride ions enter and leave the cells lining the lungs, pancreas, sweat glands, and small intestine. In people with cystic fibrosis, malfunctioning (or absent) CFTR prevents chloride from entering or leaving cells, resulting in production of a thick, sticky mucus that clogs ducts or