The Molecular Biology Of Cystic Fibrosis

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due to vas deferens obliteration, and subclinical cirrhosis of the liver . However, as the severity of these symptoms varies in different patients, and because of the presence of normal lungs after birth, it is difficult to make an early accurate prognosis (Bunch 95). The presence of mucus in the lungs provides a site where bacteria or viruses are easily caught, but which are not removed from the lung by the action of the bronchial cilliary epithelium (Figure 1).


Figure 1 - In CF the mucous layer becomes so viscous that
the cilia can no longer move it.

This leads to supradded infections. Progressive airway obstruction and inflammation causes hypoxia, a reduced lung vital capacity, and eventually over months or years hypercapnia (chronic respiratory failure) which usually leads to death (CF Foundation 1).

THE BIOCHEMISTRY OF CYSTIC FIBROSIS

It has been mentioned that the basic defect in cystic fibrosis is due to abnormalities in the secretions of the exocrine glands. ...

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ions affects the osmotic movement of water into the airway by decreasing it and thereby increasing the viscosity of the mucous secretions (Tomich 5).

Figure 2 - One proposed scheme of chloride, sodium ion and
watertransport across epithelium.

The abnormal negative potential is caused by the defective protein which is the chloride ion channel called cystic fibrosis transmembrane conductance regulator (CFTR) .
The CFTR protein is encoded by a gene , approximately 230 kb of DNA long with 27 exons, which is located on the long arm of chromosome 7. CF is caused by a defect in this gene and while many different defects cause CF, a lot of these seem to be race ...

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