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Cystic fibrosis is a life threatening, inherited disease of the exocrine glands. The condition primarily affects the digestive and respiratory systems which become clogged with a thick, sticky mucus.

Cystic fibrosis is caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene which results in an excess of salt and water passing into cells, causing a thick, sticky mucus to build up in bodily passageways.

In the airways, this mucus causes a persistent cough, wheezing, breathlessness, and repeated lung infections. In the digestive tract, the tubes that carry digestive enzymes from the pancreas into the small intestine become blocked, preventing the proper absorption of food nutrients. This can lead to poor weight gain, intestinal blockage (particularly in newborns), and foul-smelling, greasy stools.

There is no cure for cystic fibrosis, so treatment aims to ease symptoms and make the condition easier to live with. Bronchodilators may be used to help expand the airways, antibiotics to treat chest infections and physiotherapy can help expel mucus from the lungs.
The condition is most common in white people of northern European descent and is estimated to occurs in 1 in every 2,500 babies born in the UK, where babies are screened at birth for cystic fibrosis as part of the National Health Service newborn screening programme.
Special issue of Future Science OA highlights research on protein misfolding diseases

Special issue of Future Science OA highlights research on protein misfolding diseases

Future Science Group today announced the publication of a special issue in Future Science OA, covering the rapidly evolving field of protein misfolding diseases. [More]
Researchers explore better way to address the root cause of cystic fibrosis

Researchers explore better way to address the root cause of cystic fibrosis

Treatments for cystic fibrosis (CF) have added years to the lives of thousands of Americans. But they can be difficult to administer, and most don't fix the underlying cause. Scientists have now found that a small molecule, when tested in yeast, can substitute for a protein and restore a key cellular function related to those missing in people with CF and similar conditions. [More]
NIH grants Clinical and Translational Science Award to UC San Diego

NIH grants Clinical and Translational Science Award to UC San Diego

The Clinical and Translational Research Institute (CTRI) at University of California, San Diego has received a five-year Clinical and Translational Science Award (CTSA) for approximately $52 million from the National Center for Advancing Translational Science, part of the National Institutes of Health. [More]
U-M microbiome research may lead to new ways to prevent, fight lung infections in patients

U-M microbiome research may lead to new ways to prevent, fight lung infections in patients

With every breath you take, microbes have a chance of making it into your lungs. But what happens when they get there? And why do dangerous lung infections like pneumonia happen in some people, but not others? Researchers at the University of Michigan Medical School have started to answer these questions by studying the microbiome of the lungs - the community of microscopic organisms that are in constant contact with our respiratory system. [More]
New technology advances genome engineering

New technology advances genome engineering

In his mind, Basil Hubbard can already picture a new world of therapeutic treatments for millions of patients just over the horizon. It's a future in which diseases like muscular dystrophy, cystic fibrosis and many others are treated permanently through the science of genome engineering. [More]
First biodegradable gene delivery system efficiently penetrates human airway mucus barrier of lung tissue

First biodegradable gene delivery system efficiently penetrates human airway mucus barrier of lung tissue

Researchers at the Johns Hopkins University School of Medicine, Johns Hopkins University Department of Chemical and Biomolecular Engineering, and Federal University of Rio de Janeiro in Brazil have designed a DNA-loaded nanoparticle that can pass through the mucus barrier covering conducting airways of lung tissue — proving the concept, they say, that therapeutic genes may one day be delivered directly to the lungs to the levels sufficient to treat cystic fibrosis (CF), chronic obstructive pulmonary disease, asthma and other life-threatening lung diseases. [More]
New research reveals how DNA influences severity of any genetic disease

New research reveals how DNA influences severity of any genetic disease

That two people with the same disease-causing mutation do not get sick to the same extent has been puzzling scientists for decades. Now Professor Andy Fraser and his team have uncovered a key part of what makes every patient different. [More]
Canadian researchers discover how HIV evades the body's antiviral responses

Canadian researchers discover how HIV evades the body's antiviral responses

A Canadian research team at the IRCM in Montreal, led by molecular virologist Eric A. Cohen, PhD, made a significant discovery on how HIV escapes the body's antiviral responses. The team uncovered how an HIV viral protein known as Vpu tricks the immune system by using its own regulatory process to evade the host's first line of defence. [More]
Study shows possibility of using embryonic stem cells to repair damaged lung tissue

Study shows possibility of using embryonic stem cells to repair damaged lung tissue

Collectively, such diseases of the airways as emphysema, bronchitis, asthma and cystic fibrosis are the second leading cause of death worldwide. More than 35 million Americans alone suffer from chronic respiratory disease. Weizmann Institute scientists have now proposed a new direction that could, in the future, lead to the development of a method for alleviating some of their suffering. [More]
Two antioxidant supplements effective in treating mice with skin-picking disorder

Two antioxidant supplements effective in treating mice with skin-picking disorder

Two antioxidant supplements are effective in treating skin-picking disorder in mice, according to a study led by a Stanford University School of Medicine researcher. [More]
International research team identifies new gene associated with 4H leukodystrophy

International research team identifies new gene associated with 4H leukodystrophy

Leukodystrophies are deadly neurodegenerative diseases that affect one in 7,000 children and remain incurable. These genetic diseases attack myelin or the "insulating rubber sheath" surrounding neurons, which leads to deteriorating health for affected children. [More]
Celtaxsys receives FDA clearance to begin acebilustat Phase 2 trial in adult CF patients in the US

Celtaxsys receives FDA clearance to begin acebilustat Phase 2 trial in adult CF patients in the US

Celtaxsys, Inc., a clinical stage pharmaceutical development company focusing on anti-inflammatory therapeutics, including those with rare and orphan inflammatory disease indications, announced today that it has gained clearance from the U.S. Food and Drug Administration to begin a Phase 2 clinical trial of its flagship drug candidate, acebilustat (CTX-4430), in adult CF patients in the US. [More]
Gene therapy improves lung function in patients with cystic fibrosis

Gene therapy improves lung function in patients with cystic fibrosis

Gene therapy was shown to provide significant benefits for patients with cystic fibrosis in a recent clinical study published in The Lancet Respiratory Medicine journal. [More]
Gene therapy for cystic fibrosis shows significant benefit in lung function

Gene therapy for cystic fibrosis shows significant benefit in lung function

For the first time gene therapy for cystic fibrosis has shown a significant benefit in lung function compared with placebo, in a phase 2 randomised trial published in The Lancet Respiratory Medicine journal. The technique replaces the defective gene response for cystic fibrosis by using inhaled molecules of DNA to deliver a normal working copy of the gene to lung cells. [More]
Imperial College London researchers discover new inherited form of obesity, type 2 diabetes

Imperial College London researchers discover new inherited form of obesity, type 2 diabetes

Scientists have discovered a new inherited form of obesity and type 2 diabetes in humans. A large number of genes are involved in regulating body weight, and there are now over 30 genes known in which people with harmful changes in DNA sequence become extremely overweight. Similarly, there are a number of genes that can, when altered, cause type 2 diabetes. These conditions are inherited through families in exactly the same way as disorders such as cystic fibrosis or Huntington's disease. [More]
Study could point the way to new treatments for people with severe asthma

Study could point the way to new treatments for people with severe asthma

The immune response that occurs in patients with severe asthma is markedly different than what occurs in milder forms of the lung condition, according to researchers from the University of Pittsburgh School of Medicine. Those unique features could point the way to new treatments, they said in an article published online today in the Journal of Clinical Investigation. [More]

Spyryx receives award from Cystic Fibrosis Foundation to development therapeutic peptides for CF

Spyryx Biosciences, Inc., a privately-held biopharmaceutical company developing novel therapeutics for obstructive lung diseases, announced today it has received an award from Cystic Fibrosis Foundation Therapeutics, which will support development of Spyryx' therapeutic peptides for cystic fibrosis (CF). [More]
Study shows cystic fibrosis is more deadly for Hispanic than non-Hispanic patients

Study shows cystic fibrosis is more deadly for Hispanic than non-Hispanic patients

Cystic fibrosis is more deadly for Hispanic than non-Hispanic patients, a disparity that is not explained by differences in their access to health care, according to a new study from the Stanford University School of Medicine. [More]
UW microbiologist receives 2015 Shaw Prize in life science and medicine

UW microbiologist receives 2015 Shaw Prize in life science and medicine

UW microbiologist Peter Greenberg has long been fascinated with how bacteria in cells communicate for cooperative activities, including the spread of infection. He started exploring the subject in 1977, thinking the implications could be far-reaching. [More]

Loyola patient celebrates 10th anniversary of heart and double-lung transplant

On March 20, Andrew Gaumer celebrated the 10th anniversary of his life-saving heart and double-lung transplant. On Mother's Day, his wife, Andrea, gave birth to their first child, a baby boy named Jude. And on June 21, Andrew will celebrate his first Father's Day - a milestone he once feared he would never see. [More]
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