Cystic Fibrosis

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Cystic Fibrosis

What causes Cystic Fibrosis?

  • CF patients have faulty version of CFTR protein- has a different tertiary structure to that of a normal one. 
  • This means the protein cannot transport the chloride ions from the inside of the cell to the outside of the cell.
  • Instead the water potential of the mucus (outside the cell) increases and water leaves the mucus by osmosis.
  • This makes the mucus very thick and sticky making it very hard for the cilia (epithelium cell) to remove and get rid off.

The symptoms of Cystic Fibrosis 

  • Lung infections; As the mucus is very thick it is very hard for the cilia to remove it by either coughing it up or swolling it so, it just builds up inside the air passages. This means people with CF develope lung infections or coughing and wheezing very quicky. Eventually, these lung infections cause damage to the lung over time. Also, when the bacteria grow in the


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