Articles with the keyword: 


Blood tests may show inherited diseases in fetuses
sea-maid submitted, created time 6 days 12 hours (www.reuters.com)
Doctors may soon be able to diagnose inherited diseases such as cystic fibrosis, thalassaemia and sickle cell anemia in fetuses by simply testing a blood sample taken from the mother. 


Bacterial locomotion revealed! ...and stoppable!
Darkfrog submitted, created time 5 months 1 week (www.nature.com)
Bacteria have two forms, free-swimming and sessile. It is during this stationary biofilm stage that they cause the most trouble, building up on scientific and medical equipment and forming huge, multi-species colonies or worse--building up in the lungs of people with cystic fibrosis. We've known for years that bacteria have to shut down their flagella to form biofilms, but now we have an idea of how it's done. The gene in question is called epsE
The article describes the molecular motor function as more of a clutch than a brake, but we didn't have auto shop at my high school 


Lung transplants no help for cystic fibrosis!
Eric wu submitted, created time 1 year 1 week (www.reuters.com)
Children with severe cystic fibrosis are seldom helped by a lung transplant, researchers said on Wednesday in findings they said stunned them. 


Cindy submitted, created time 1 year 4 months (www.sciencedaily.com)
The structure of a novel protein in the bacterium that is the most persistent pathogen in cystic fibrosis (CF) patients has been solved. 


HWI Researchers Determine Structure of Protein from Pathogen Associated With CF and TB
bianjie submitted, created time 1 year 4 months (www.hwi.buffalo.edu)
The structure of a novel protein in the bacterium that is the most persistent pathogen in cystic fibrosis patients has been solved. 


Opportunistic infections in lung disease: Pseudomonas infections in cystic fibrosis
athena submitted, created time 1 year 6 months (www.sciencedirect.com)
"Pseudomonas aeruginosa is an opportunistic pathogen that significantly contributes to morbidity and mortality in patients with cystic fibrosis. Defective mucociliary clearance associated with the absence of the functional cystic fibrosis transmembrane conductance regulator in airway epithelium plays a critical role in the initial colonization of this pathogen. P. aeruginosa, while initiating a profound inflammatory response, employs multiple mechanisms to evade immune clearance 


SPDEF regulates goblet cell hyperplasia in the airway epithelium
athena submitted, created time 1 year 7 months (www.jci.org)
"Goblet cell hyperplasia and mucous hypersecretion contribute to the pathogenesis of chronic pulmonary diseases including cystic fibrosis, asthma, and chronic obstructive pulmonary disease" 


Drug takes no nonsense from cystic fibrosis
athena submitted, created time 1 year 7 months (www.newscientist.com)
"Some types of genetic disease, such as cystic fibrosis, may be beaten by overcoming the "nonsense" mutations that cause them, researchers say. They have now identified a drug that appears to correct this kind of genetic defect in mice." 


Inhibition of CFTR Cl- channel function caused by enzymatic hydrolysis of sphingomyelin
Luneetty submitted, created time 1 year 7 months (www.pnas.org)
Numerous mutations in the cystic fibrosis (CF) transmembrane conductance regulator (CFTR, a Cl- channel) disrupt salt and fluid transport and lead to the formation of thick mucus in patients' airways. Obstruction by mucus predisposes CF patients to chronic infections and inflammation, which become gradually harder to control and eventually fatal. Aggressive antibiotic therapy and supportive measures have dramatically lengthened CF patients' lives. Here, we report that sphingomyelinases (SMase) from human respiratory pathogens strongly inhibit CFTR function 
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