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Study sheds light on how cystic fibrosis works

Respiratory Care Weekly, June 14, 2006

Researchers report they may have found the mechanism in which cystic fibrosis (CF) makes the lungs vulnerable to pseudomonas aeruginosa (PA), according to the June 9 Science. PA is a pathogen that infects more than 80% of CF patients. Studying blood and sputum from CF patients, the researchers discovered the protein sequence Hcp1, common to most CF patients, may be the link that could allow PA to survive in CF patients' lungs. PA doesn't survive in patients with normal lungs and healthy immune systems.

"Since we know so little about what this bacterium is up to while it's engaged in these chronic infections, the discovery of this protein secretion system might lead to finding a new target for treatments," said Joseph Mougous, lead author of the study and research fellow in the Harvard Medical School Department of Microbiology and Molecular Genetics.

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