ScienceDaily: Cystic Fibrosis News

Discovery holds potential in destroying drug-resistant bacteria

Tue, 07 May 2013 15:50:50 EDT

Through the serendipity of science, researchers have discovered a potential treatment for deadly, drug-resistant bacterial infections that uses the same approach that HIV uses to infect cells. New research is especially promising in the development of a potential treatment for lung infections in people with cystic fibrosis.

A protein's well-known cousin sheds light on its gout-linked relative

Mon, 08 Apr 2013 10:32:32 EDT

A genetic mutation causes a breakdown in a cellular pump that clears uric acid from the blood. By comparing this protein pump to a related protein involved in cystic fibrosis, the researchers also identified a compound that partially repairs the pump.

Rise in CF patient infections explained: DNA sequencing reveals evidence for Mycobacterium abscessus transmission between Cystic Fibrosis patients

Fri, 29 Mar 2013 09:03:03 EDT

Whole genome sequencing has explained why infection by the multidrug resistant bacteria, Mycobacterium abscessus, has been on the increase in Cystic Fibrosis patients. This study revealed that frequent transmission of the bacteria occurs between CF patients despite conventional cross-infection measures.

Discovery could increase efficacy of promising cystic fibrosis drug

Thu, 21 Mar 2013 15:15:15 EDT

Researchers believe they have found exactly how Kalydeco (Vx-770), the first drug of its kind to treat the underlying cause of cystic fibrosis, works and how to improve its effectiveness in the future.

Potential new target in fight against 'superbug'

Wed, 30 Jan 2013 08:30:30 EST

Researchers have discovered how an antibiotic-resistant superbug exploits oxygen-limited conditions in the lungs of patients with severe respiratory disease to thrive.

New strategy to fight deadly infection in cystic fibrosis

Wed, 23 Jan 2013 11:42:42 EST

New research suggests that lowering excessive levels of a protein in immune system cells could be a strategy to clear an infection that is deadly to patients with cystic fibrosis (CF).

Impaired blood vessel function found in cystic fibrosis patients

Mon, 26 Nov 2012 11:07:07 EST

The first evidence of blood vessel dysfunction has been found in a small cohort of generally healthy young people with cystic fibrosis, researchers report.

New drug target found for cystic fibrosis lung disease

Wed, 07 Nov 2012 13:29:29 EST

Researchers have discovered the cellular pathway that causes the lung-damaging inflammation in cystic fibrosis. The study also showed that reducing the pathway's activity decreases inflammation. The finding offers a potential new drug target for treating CF lung disease, which is a major cause of illness and death for people with CF.

Insights into new therapy for rare form of cystic fibrosis

Mon, 29 Oct 2012 08:24:24 EDT

Scientists have established that a drug recently approved by the US Food and Drug Administration to treat a rare form of cystic fibrosis works in an unconventional way. Their results reveal new possibilities for treating various forms of cystic fibrosis.

New insights into functionality of cystic fibrosis protein

Wed, 26 Sep 2012 12:38:38 EDT

CFTR is an important protein that, when mutated, causes the life-threatening genetic disease cystic fibrosis. A new study details how an accidental discovery has provided new understanding about CFTR functionality.

Parents of babies with sickle cell trait are less likely to receive genetic counseling

Tue, 11 Sep 2012 12:49:49 EDT

Parents of newborns with the sickle cell anemia trait were less likely to receive genetic counseling than parents whose babies are cystic fibrosis carriers, a new study shows.

Human lungs brush out intruders

Thu, 23 Aug 2012 14:30:30 EDT

A new study helps to explain how human airways clear mucus out of the lungs. The findings may give researchers a better understanding of what goes wrong in many human lung diseases, such as cystic fibrosis, chronic obstructive pulmonary disease and asthma.

Gene network restores cystic fibrosis protein function

Wed, 01 Aug 2012 09:38:38 EDT

Researchers have discovered a genetic process that can restore function to a defective protein, which is the most common cause of cystic fibrosis.

New proteins to clear the airways in cystic fibrosis and COPD

Fri, 13 Jul 2012 12:29:29 EDT

Scientists discovered a new strategy to help CF and COPD patients clear the thick and sticky mucus clogging their lungs, leading to life-threatening infections. The report shows the "SPLUNC1" protein and its derivative peptides may help thin mucus by affecting the epithelial sodium channel. Along with its implications for cystic fibrosis and COPD, the research enhances the understanding of hypertension due to its role in controlling blood pressure.

Exome sequencing of health condition extremes can reveal susceptibility genes

Sun, 08 Jul 2012 16:23:23 EDT

Comparing DNA from patients at the best and worst extremes of a health condition can reveal genes for resistance and susceptibly. This approach discovered rare variations in the DCTN4 gene among cystic fibrosis patients prone to early, chronic airway infections. The gene codes for part of a molecular motor that moves microbes along a cellular conveyer belt to their annihilation. Similar "testing the extremes" strategies may uncover genes behind more common traits -- healthy and unhealthy hearts, leaness and obesity, and normal and high blood pressure.

Cystic fibrosis breakthrough reveals why females fare worse than males

Thu, 24 May 2012 09:27:27 EDT

Researchers have discovered why females with cystic fibrosis do worse than males. The study is the first to show that the female hormone estrogen promotes the presence of a particular form of bacteria which results in more severe symptoms for female cystic fibrosis patients. In addition, females who were taking the oral contraceptive pill, which decreases the amount of naturally occurring estrogen in their bodies, were found to have lower levels of the problematic bacteria.

Concentrated saline therapy not effective in young children with cystic fibrosis

Mon, 21 May 2012 10:46:46 EDT

Inhaling concentrated saline (salt water) mist does not reduce how often infants and young children with cystic fibrosis need antibiotics for respiratory symptoms, according to new findings. This trial is the largest study of concentrated, or hypertonic, saline therapy in infants and preschoolers.

Why one bacterial infection is so deadly in cystic fibrosis patients: Pathogen interferes with cells whose job is to fight infection

Sun, 22 Apr 2012 16:24:24 EDT

The bacterium Burkholderia cenocepacia is harmless in healthy people but causes a severe and persistent lung infection in cystic fibrosis patients and is resistant to nearly all known antibiotics. The bacterium interferes with an important survival process in cells whose job is to fight infection. This phenomenon is even stronger in CF patients, new research shows.

Computer-designed molecules point to new therapy for cystic fibrosis

Thu, 19 Apr 2012 19:17:17 EDT

By developing software that uses 3-D models of proteins involved in cystic fibrosis, a team of scientists has identified several new molecules that may ease the symptoms of the disease.

Gene variations linked to intestinal blockage in newborns with cystic fibrosis

Sun, 01 Apr 2012 13:49:49 EDT

The discovery by cystic fibrosis researchers offers the possibility of developing therapies to intervene in utero. Some of these genes may influence disease in other cystic fibrosis-affected organs.

'Two steps' ahead in cystic fibrosis research

Mon, 12 Mar 2012 11:41:41 EDT

Restoring normal function to the mutant gene product responsible for cystic fibrosis (CF) requires correcting two distinct structural defects, according to new research. This finding could point to more effective therapeutic strategies for CF in the future.

FDA approves new drug for cystic fibrosis

Tue, 31 Jan 2012 17:18:18 EST

The FDA's approval of ivacaftor for treatment of cystic fibrosis will provide substantial benefit for CF patients with the G551D mutation in CFTR, say investigators who conducted clinical trials on the drug.

New medication effectively treats underlying cause of cystic fibrosis in some patients, study shows

Wed, 02 Nov 2011 19:04:04 EDT

A new study has confirmed that the drug, ivacaftor (VX-770), significantly improves lung function in some people with cystic fibrosis. The oral medicine targets the defective protein produced by the gene mutation called G551D that causes CF. Researchers found that patients carrying G551D -- approximately four per cent of all CF patients -- who were treated with VX-770 showed a 17 per cent relative improvement in lung function that was sustained over the course of 48 weeks.

Smoking cigarettes simulates cystic fibrosis

Wed, 12 Oct 2011 11:33:33 EDT

If you smoke cigarettes, you have more in common with someone who has cystic fibrosis than you think. A new research report shows that smoking cigarettes affects the lungs in a way that is very similar to cystic fibrosis, a life threatening disease affecting the lungs and other organs.

Progression of lung fibrosis blocked in mouse model

Wed, 05 Oct 2011 17:26:26 EDT

A new study may lead to a way to prevent the progression, or induce the regression, of lung injury that results from use of the anti-cancer chemotherapy drug bleomycin. Pulmonary fibrosis caused by this drug, as well as idiopathic pulmonary fibrosis from unknown causes, affect nearly five million people worldwide. No therapy is known to improve the health or survival of patients.

Unraveling a new regulator of cystic fibrosis

Mon, 19 Sep 2011 11:38:38 EDT

Cystic fibrosis is caused by a genetic defect in a chloride channel called cystic fibrosis transmembrane conductase regulator. Although scientists do not fully understand how or why this defect occurs, researchers have found a promising clue: a protein called ubiquitin ligase Nedd4L.

Cellular intricacies of cystic fibrosis

Mon, 19 Sep 2011 10:48:48 EDT

When researchers discovered the primary genetic defect that causes cystic fibrosis (CF) back in 1989, they opened up a new realm of research into treatment and a cure for the disease. Since then, scientists have been able to clone the defective gene and study its effects in animals. Now researchers have developed a technique for observing the defects at work in human tissue donated by patients with CF.

An 'unconventional' path to correcting cystic fibrosis

Thu, 01 Sep 2011 13:46:46 EDT

Researchers have identified an unconventional path that may correct the defect underlying cystic fibrosis, according to a new study. This new treatment dramatically extends the lives of mice carrying the disease-associated mutation.

Why long-term antibiotic use increases infection with a mycobacterium

Mon, 01 Aug 2011 12:29:29 EDT

The clinical outcome is improved if patients with chronic lung diseases such as cystic fibrosis are treated long-term with the antibiotic azithromycin. However, azithromycin treatment in patients with cystic fibrosis as recently associated with increased infection with nontuberculous mycobacteria. Now, researchers have confirmed that long-term use of azithromycin by adults with cystic fibrosis is associated with infection with nontuberculous mycobacteria and identified an underlying mechanism.

Invasive diagnostic procedure for children with cystic fibrosis does not improve outcomes, study finds

Tue, 12 Jul 2011 16:28:28 EDT

Compared to a standard diagnostic procedure, infants with cystic fibrosis who received treatment based on a diagnostic procedure involving obtaining and culturing fluid samples from the lungs did not have a lower prevalence of lung-damaging infection or structural lung injury at 5 years of age, according to a new study.

Stem cell scientists discover new airway stem cell

Mon, 27 Jun 2011 18:39:39 EDT

Researchers have identified a new stem cell that participates in the repair of the large airways of the lungs, which play a vital role in protecting the body from infectious agents and toxins in the environment.

First ever drug to treat ‘Celtic Gene’ in cystic fibrosis sufferers

Mon, 20 Jun 2011 09:52:52 EDT

Scientists have developed a new treatment for cystic fibrosis. The new drug will benefit sufferers who have the ‘Celtic Gene’, a genetic mutation which is particularly common in Ireland.

Cystic fibrosis-associated bacteria could help fight back against antibiotic resistance

Fri, 27 May 2011 10:12:12 EDT

A bacteria which infects people with cystic fibrosis could help combat other antibiotic-resistant microbes, according to new research. Scientists have discovered antibiotics from Burkholderia are effective against MRSA and even other cystic fibrosis infecting bacteria.

Genome regions that could influence severity of cystic fibrosis identified

Mon, 23 May 2011 12:13:13 EDT

Scientists have pinpointed regions of the genome that contribute to the debilitating lung disease that is the hallmark of cystic fibrosis.

Simple exercise improves lung function in children with cystic fibrosis

Tue, 03 May 2011 13:26:26 EDT

A small study of children and teens with cystic fibrosis (CF) shows that simple exercise, individually tailored to each patient's preference and lifestyle, can help improve lung function and overall fitness.

Video games may help clear airway of cystic fibrosis patients

Sat, 30 Apr 2011 13:31:31 EDT

Video games controlled by the player's breath can encourage youths with cystic fibrosis to use techniques that can help keep their airways clearer, according to a new study.

Combination therapy provides hope for cure of dangerous infections of cystic fibrosis patients

Sun, 24 Apr 2011 15:24:24 EDT

An over-the-counter drug used to treat diarrhea combined with minocycline, an antibiotic used to treat bacterial infections, could one day change the lives of those living with cystic fibrosis.

Pig model of cystic fibrosis improves understanding of disease

Wed, 16 Mar 2011 15:29:29 EDT

Using a newly created pig model that genetically replicates the most common form of cystic fibrosis, researchers have now shown that the CF protein is "misprocessed" in the pigs and does not end up in the correct cellular location. This glitch leads to disease symptoms, including gastrointestinal abnormalities and lung disease in the pigs, which mimic CF in humans.

Scientists to sequence DNA of cystic fibrosis superbug

Tue, 22 Feb 2011 09:26:26 EST

Scientists in the UK are using the latest DNA sequencing technology to understand the diversity of a bacterium that causes severe lung infection in cystic fibrosis patients.

New method attacks bacterial infections on contact lenses

Tue, 25 Jan 2011 16:08:08 EST

Researchers have discovered a new method to fight bacterial infections associated with contact lenses. The method may also have applications for bacterial infections associated with severe burns and cystic fibrosis.

Protein involved in cystic fibrosis also plays role in emphysema, chronic lung disease

Wed, 29 Dec 2010 14:21:21 EST

Researchers have discovered that a protein involved in cystic fibrosis also regulates inflammation and cell death in emphysema and may be responsible for other chronic lung diseases. The findings pave the way toward new treatments to prevent lung damage caused by infections or cigarette smoke in emphysema.

Novel drug offers hope for early intervention in cystic fibrosis patients

Fri, 17 Dec 2010 14:56:56 EST

Cystic fibrosis (CF) patients with normal to mildly impaired lung function may benefit from a new investigational drug designed to help prevent formation of the sticky mucus that is a hallmark of the disease, according to researchers involved in a phase 3 clinical trial of the drug. Called denufosol, the investigational medication can be given early in the CF disease process, and may help delay the progression of lung disease in these patients, the researchers found.

Novel approach shows promise for cystic fibrosis

Wed, 17 Nov 2010 18:44:44 EST

An investigational drug targeting a defective protein that causes cystic fibrosis has been shown to improve lung function in a small study of CF patients. The drug, VX-770, appeared to improve function of what is known as CFTR -- the faulty protein responsible for CF. It is among the first compounds being developed for CF that specifically targets the root cause of cystic fibrosis.

Common strain of bacteria found in patients with cystic fibrosis in Canada; associated with greater risk of death

Tue, 16 Nov 2010 18:19:19 EST

A common transmissible strain of the bacteria Pseudomonas aeruginosa has been identified among cystic fibrosis (CF) patients in Canada, suggesting that cross-infection has occurred widely between CF centers in the United Kingdom and Canada, according to a new study. Infection with this strain among Canadian CF patients has been associated with an increased risk of death or lung transplantation.

Cystic fibrosis gene typo is a double whammy

Sat, 13 Nov 2010 16:54:54 EST

Researchers have demonstrated that the gene mutated in cystic fibrosis not only controls traffic on the chloride highway, but also keeps the sodium highway from being overused.

Human induced pluripotent stem cells generated to further treatments for lung disease

Thu, 28 Oct 2010 11:36:36 EDT

Researchers have generated 100 new lines of human induced pluripotent stem cells (iPSC) from individuals with lung diseases, including cystic fibrosis and emphysema. The new stem cell lines could possibly lead to new treatments for these debilitating diseases. The findings demonstrate the first time lung disease-specific iPSC have been created in a lab.

Step closer to drug treatment for cystic fibrosis?

Tue, 12 Oct 2010 11:40:40 EDT

New research may move scientists closer to a cure for cystic fibrosis, one of the most common fatal genetic diseases.

Channeling efforts to fight cystic fibrosis: Crosstalk between ion channels points to new therapeutic strategy

Fri, 17 Sep 2010 15:18:18 EDT

Researchers have found a possible new target for fighting cystic fibrosis (CF) that could compensate for the lack of a functioning ion channel in affected CF-related cells.

Older patients offer insight into the future of cystic fibrosis

Thu, 20 May 2010 09:29:29 EDT

An emerging population of middle-aged cystic fibrosis patients contains significantly more females and includes a large proportion of patients who lived for decades without a diagnosis or specialized care, according to new research.

Pigs provide clues on cystic fibrosis lung disease

Wed, 28 Apr 2010 14:22:22 EDT

Aided by a new experimental model, scientists are a step closer to understanding how cystic fibrosis (CF) causes lung disease in people with the condition. The findings could help improve treatments for lung disease, which causes most of the deaths and disability among people with CF.

New hope exists in treating inherited disease by suppressing DNA mutations

Mon, 26 Apr 2010 13:14:14 EDT

Genetic mutation can disrupt the way human cells make proteins, which in turn leads to inherited disease. According to one researcher, scientists are closer than ever to producing drugs that fix this disrupted-protein pathway and drastically improving treatment of genetic disease.

Cystic fibrosis and Crohn's disease treated successfully with infliximab

Mon, 19 Apr 2010 10:29:29 EDT

A research team from Italy reported a case of a 23-year-old patient suffering from cystic fibrosis (CF) and Crohn's disease who was successfully treated with infliximab. This case report was thought to be one of the first regarding the use of biological therapy in patients with CF.

Molecular basis for Pseudomonas aeruginosa persistent infections in CF patients

Thu, 11 Mar 2010 20:27:27 EST

New research reveals Small Colony Variants (SCVs) of P. aeruginosa to be a hallmark of chronic infection in cystic fibrosis (CF) patients. Results suggest that SCV-mediated persistence might be a good target for antimicrobial chemotherapy.

Defective signaling pathway sheds light on cystic fibrosis

Sun, 14 Feb 2010 14:31:31 EST

In a study that could lead to new therapeutic targets for patients with the cystic fibrosis, scientists have identified a defective signaling pathway that contributes to disease severity.

Adapting to clogged airways makes common pathogen resist powerful drugs

Tue, 09 Feb 2010 18:32:32 EST

Mutations of a common environmental pathogen that causes chronic lung disease in patients with cystic fibrosis are able to survive in oxygen-poor, nitrate-rich airway secretions. This same survival mechanism also enables the mutate bacteria to resist the effects of certain antibiotics -- even without any previous exposure to antibiotics.

Biodegradable particles can bypass mucus, release drugs over time

Mon, 04 Jan 2010 11:45:45 EST

Researchers have created biodegradable nanosized particles that can easily slip through the body's sticky and viscous mucus secretions to deliver a sustained-release medication cargo.

Fight infection by disturbing how bacteria communicate

Mon, 21 Dec 2009 13:02:02 EST

Researchers have clarified the structure of an enzyme that disturbs the communication processes between bacteria. By doing so they have laid the foundations for a new method of tackling bacterial infections such as cystic fibrosis.

Better understanding of the defective protein that causes cystic fibrosis

Fri, 18 Dec 2009 08:35:35 EST

Scientists studying the protein that, when defective or absent, causes cystic fibrosis has made an important discovery about how that protein is normally controlled and under what circumstances it might go awry.

Scientists restore some function to cells from cystic fibrosis patients

Sun, 06 Dec 2009 16:29:29 EST

In an encouraging new development, scientists have restored partial function to lung cells collected from patients with cystic fibrosis. While there is still much work to be done before the therapy can be tested in humans, the discovery opens the door to a new class of therapies for this and a host of other chronic diseases.

Lung Tissue Generated From Human Embryonic Stem Cells

Wed, 04 Nov 2009 19:18:18 EST

Scientists in Belgium have successfully differentiated human embryonic stem cells (hESC) into major cell types of lung epithelial tissue using a convenient air-liquid interface. The technique could provide an alternative to lung transplants for patients with lung injury due to chronic pulmonary disease and inherited genetic diseases such as cystic fibrosis.