Articles tagged with Cystic Fibrosis
Dr. Boucher is a leading researcher on cystic fibrosis, publishing over 300 articles and developing novel treatments. The University of North Carolina's genomics initiative receives $245 million in funding to advance research and therapies.
A clearer picture has been obtained on why the protein doesn't function properly in the milder form of cystic fibrosis, attributed to segments sticking together and interfering with chloride flow. This discovery opens doors for new avenues of research in rational drug design.
Jayraman et al. used fluorescent indicators to measure ASL salinity in normal human and CFTR-/- mice, finding no significant difference between the two groups. This noninvasive approach may provide new insights into lung diseases with poorly understood ASL properties.
Researchers at Johns Hopkins have identified a previously unknown system that helps control the protein product made by the cystic fibrosis gene, called CFTR. The discovery could lead to improved drug testing and screening for cystic fibrosis therapies.
A team of researchers has completed the genome sequence of Pseudomonas aeruginosa, the largest bacterium sequenced so far. The findings may lead to innovative therapies for patients with cystic fibrosis and those who develop severe infections.
Researchers at UNC-CH have successfully repaired a genetic problem causing cystic fibrosis, offering new hope for patients. The technique involves correcting a mutation involving unnecessary information inside a gene, resulting in the production of normal messenger RNA.
Researchers have discovered a reversible lipid imbalance that may be responsible for the common symptoms of cystic fibrosis. Administration of DHA corrected the imbalance and reversed signs of cystic fibrosis in mice with the genetic defect.
A University of Iowa study reveals that high salt concentrations in the airways of people with cystic fibrosis prevent antimicrobial peptides from working effectively. This finding provides new ideas about potential treatments for the disease.
Researchers have discovered that movement of salt and liquid in and out of lung surface cells plays a central role in regulating the lungs' internal liquid coating. This finding confirms a theory of how lungs protect themselves and may suggest better ways of treating cystic fibrosis.
Scientists have extended their gene repair technology to correct point and frameshift genetic mutations, with potential applications for treating cystic fibrosis and other diseases caused by DNA deletions.
A study found that a significant number of infertile men have cystic fibrosis gene mutations undetected by routine screening. This raises concerns about the risk of passing on defective genes to future generations. The researchers suggest improving screening methods for men and testing women for gene mutations to mitigate this risk.
Cystic fibrosis damages young lungs due to chronic bacterial infections and a lack of liquid layer, which hinders the body's natural cleansing process. Researchers have discovered that the film of liquid coating airways is depleted in CF patients, preventing cilia from functioning properly.
Researchers at UNC-CH found that Pamidronate is highly effective in partially reversing bone loss caused by immuno-suppressant drugs after lung transplants. Patients treated with the drug experienced a 10% reversal of bone loss, and showed significant increases in femur and spine mineral densities.
Researchers found a strong association between the cystic fibrosis gene and chronic pancreatitis, revealing genetics plays a significant role in its development. The study's findings have significant implications for treating patients with this condition, including those who are not heavy drinkers.
A shellfish toxin study has discovered a mechanism that may help treat cystic fibrosis by stimulating fluid flow through protein phosphatase inhibition. This could lead to loosening of accumulated mucus in the lungs and gut, reducing bacterial infections and early death.
A bacterial pesticide, Burkholderia cepacia, has raised concerns over its potential to cause deadly lung infections in people with cystic fibrosis. Experts are calling for a ban on its use in pest control until it is proven safe.
A new study reveals that many laboratories offering genetic tests for cystic fibrosis don't test for enough mutations, which can lead to unreliable results. The recommended widespread screening is premature due to the lack of standardization in testing labs.
A study led by Johns Hopkins Researchers found that a drug, sodium 4-phenylbutyrate (4PBA), may help cystic fibrosis patients with the deltaF508 mutation by allowing more CFTR proteins to reach cell surfaces. This phenomenon occurs at concentrations normally seen in patients taking the drug for urea cycle disorders.
Researchers at UNC-CH School of Medicine found a genetic mutation, 5T allele, associated with milder forms of cystic fibrosis and other chronic lung and sinus infections. The study suggests that the 5T allele may be linked to infertility and urological abnormalities in infertile men.