Cystic Fibrosis - Research Article from Macmillan Science Library: Genetics

This encyclopedia article consists of approximately 6 pages of information about Cystic Fibrosis.
This section contains 1,630 words
(approx. 6 pages at 300 words per page)
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Clinical Features

CF is a multisystem disease, affecting a number of different organs and tissues throughout the body. Most of these manifestations have in common the production of abnormally viscous secretions from glands and surface epithelial cells. In the lung the mucus secretions from the bronchial epithelial cells are unusually thick. They are difficult to clear properly from the airway passages and, instead, tend to collect and obstruct the bronchial tree, while providing a perfect culture medium for dangerous bacteria. Over time, repeated bacterial infections damage and destroy the lung tissue, leading to chronic breathing problems and, eventually, to the loss of viable lung function. Indeed, the pulmonary manifestations of the disease are the main cause of death in most CF patients.

There are three types of defects in CFTR production that can cause cystic fibrosis. The most common mutation, ΔF508, causes faulty protein processing and degradation of the protein before it reaches the cell membrane. Other defects are also possible. There are three types of defects in CFTR production that can cause cystic fibrosis. The most common mutation, ΔF508, causes faulty protein processing and degradation...

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This section contains 1,630 words
(approx. 6 pages at 300 words per page)
Buy the Cystic Fibrosis Encyclopedia Article
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Macmillan Science Library: Genetics
Cystic Fibrosis from Macmillan Science Library: Genetics. Copyright © 2001-2006 by Macmillan Reference USA, an imprint of the Gale Group. All rights reserved.
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