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yet</button><a id="jr-fip-next" class="wsprkl btn" title="Jump to next match">▶</a></nav></nav></div><div id="jr-epub-interstitial" class="hidden"></div><div id="jr-content"><article data-type="main"><div class="main-content lit-style" itemscope="itemscope" itemtype="http://schema.org/CreativeWork"><div class="meta-content fm-sec"><div class="fm-sec"><h1 id="_NBK22202_"><span class="title" itemprop="name">Cystic fibrosis</span></h1><p class="fm-aai"><a href="#_NBK22202_pubdet_">Publication Details</a></p></div></div><div class="body-content whole_rhythm" itemprop="text"><p>Cystic fibrosis (CF) is the most common fatal genetic disease in the United States today. It causes the body to produce a thick, sticky mucus that clogs the lungs, leading to infection, and blocks the pancreas, stopping digestive enzymes from reaching the intestines where they are required to digest food.</p><div class="iconblock whole_rhythm clearfix ten_col fig" id="figCFFigure" co-legend-rid="figlgndCFFigure"><a href="/books/NBK22202/figure/CFFigure/?report=objectonly" target="object" title="Building mouse models of human disease" class="img_link icnblk_img figpopup" rid-figpopup="figCFFigure" rid-ob="figobCFFigure"><img class="small-thumb" src="/books/NBK22202/bin/CF.gif" src-large="/books/NBK22202/bin/CF.jpg" alt="Building mouse models of human disease. Expression of a human cystic fibrosis (CFTR) gene in the gut of a mouse." /></a><div class="icnblk_cntnt" id="figlgndCFFigure"><h4 id="CFFigure"><a href="/books/NBK22202/figure/CFFigure/?report=objectonly" target="object" rid-ob="figobCFFigure">Building mouse models of human disease</a></h4><p class="float-caption no_bottom_margin">Expression of a human cystic fibrosis (CFTR) gene in the gut of a mouse. A human antisense probe was used to show human CFTR expressed in the mouse duodenum. Image reproduced with permission from Manson, A.L et al. (1997) EMBO J. 16, 4238-4249.</p></div></div><p>CF is caused by a defective gene, which codes for a chloride transporter found on the surface of the epithelial cells that line the lungs and other organs. Several hundred mutations have been found in this gene, all of which result in defective transport of chloride, and secondarily sodium, by epithelial cells. As a result, the amount of sodium chloride (salt) is increased in bodily secretions. The severity of the disease symptoms of CF is directly related to the characteristic effects of the particular mutation(s) that have been inherited by the sufferer.</p><p>CF research has accelerated sharply since the discovery of CFTR in 1989. In 1990, scientists successfully cloned the normal gene and added it to CF cells in the laboratory, which corrected the defective chloride transport mechanism. This technique—gene therapy—was then tried on a limited number of CF patients. However, this treatment may not be as successful as originally hoped. Further research will be required before gene therapy, and other experimental treatments, prove useful in combating CF.
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</p><div id="cysticfibrosis.disease-categories"><h2 id="_cysticfibrosis_disease-categories_">Related diseases</h2><ul class="simple-list"><li class="half_rhythm"><div>
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<a href="/books/n/gnd/A25/?report=reader">See other Diseases of the Digestive System</a>
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</div></li><li class="half_rhythm"><div>
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<a href="/books/n/gnd/A65/?report=reader">See other Neonatal Diseases</a>
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</div></li><li class="half_rhythm"><div>
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<a href="/books/n/gnd/A88/?report=reader">See other Respiratory Diseases</a>
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</div></li></ul></div><div id="bk_toc_contnr"></div></div></div><div class="fm-sec"><h2 id="_NBK22202_pubdet_">Publication Details</h2><h3>Publication History</h3><p class="small">Last Update: <span itemprop="dateModified">January 31, 2011</span>.</p><h3>Copyright</h3><div><div class="half_rhythm"><a href="/books/about/copyright/">Copyright Notice</a></div></div><h3>Publisher</h3><p><a href="https://www.ncbi.nlm.nih.gov/" ref="pagearea=page-banner&targetsite=external&targetcat=link&targettype=publisher">National Center for Biotechnology Information (US)</a>, Bethesda (MD)</p><h3>NLM Citation</h3><p>National Center for Biotechnology Information (US). Genes and Disease [Internet]. Bethesda (MD): National Center for Biotechnology Information (US); 1998-. Cystic fibrosis. [Updated 2011 Jan 31].<span class="bk_cite_avail"></span></p></div><div class="small-screen-prev"><a href="/books/n/gnd/crohnsdisease/?report=reader"><svg xmlns="http://www.w3.org/2000/svg" viewBox="0 0 100 100" preserveAspectRatio="none"><path d="M75,30 c-80,60 -80,0 0,60 c-30,-60 -30,0 0,-60"></path><text x="20" y="28" textLength="60" style="font-size:25px">Prev</text></svg></a></div><div class="small-screen-next"><a href="/books/n/gnd/deafness/?report=reader"><svg xmlns="http://www.w3.org/2000/svg" viewBox="0 0 100 100" preserveAspectRatio="none"><path d="M25,30c80,60 80,0 0,60 c30,-60 30,0 0,-60"></path><text x="20" y="28" textLength="60" style="font-size:25px">Next</text></svg></a></div></article><article data-type="fig" id="figobCFFigure"><div id="CFFigure" class="figure bk_fig"><div class="graphic"><img data-src="/books/NBK22202/bin/CF.jpg" alt="Building mouse models of human disease. Expression of a human cystic fibrosis (CFTR) gene in the gut of a mouse." /></div><h3><span class="label">Building mouse models of human disease</span></h3><div class="caption"><p>Expression of a human cystic fibrosis (CFTR) gene in the gut of a mouse. A human antisense probe was used to show human CFTR expressed in the mouse duodenum. </p><p>Image reproduced with permission from Manson, A.L et al. (1997) EMBO J. 16, 4238-4249.</p></div></div></article></div><div id="jr-scripts"><script src="/corehtml/pmc/jatsreader/ptpmc_3.22/js/libs.min.js"> </script><script src="/corehtml/pmc/jatsreader/ptpmc_3.22/js/jr.min.js"> </script></div></div>
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