Navigation Links
Penn study on lung-infecting bacterial enzyme suggests new approach to cystic fibrosis treatment

Researchers at the University of Pennsylvania School of Medicine have discovered that an enzyme produced by lung-infecting bacteria further shuts down a protein that is defective in cystic fibrosis patients. The disruption to this protein that conveys ions from lung cells to airways causes thick mucus to buildup inside the lung. The finding suggests a new therapeutic target for treating lung infections in some cystic fibrosis (CF) patients.

Lung infection, facilitated by CF mutations, is the main cause of death in CF patients. This bacterial component to CF now helps explain why the severity of CF symptoms did not match the pathological effect of the CF mutation alone. The study was published this month in the Proceedings of the National Academy of Sciences.

The research, conducted by Zhe Lu, MD, PhD; Yajamana Ramu, PhD; and Yanping Xu, MD, PhD, of the Department of Physiology, shows that the bacterial enzyme, called sphingomyelinase (SMase), disables a protein in lungs called CFTR, for cystic fibrosis transmembrane conductance regulator. SMase is made by the bacteria that cause pneumonia, some anthrax-causing bacteria, and bacteria that cause opportunistic infections in CF and AIDS patients.

In healthy lungs, CFTR allows the passage of chloride ions (and accompanying water) into airways, creating a thin layer of fluid to keep airways clear. However, SMase, secreted by certain respiratory tract bacteria, breaks down lipids surrounding CFTR and thereby suppresses CFTR’s chloride-passing function. To make matters worse, the products of the lipid breakdown are also known to trigger inflammation and cell death.

Together, these facts compellingly suggest that SMase plays a critical role in the heretofore mysterious pathogenesis of lung injury in CF patients. They also present a new paradigm for treating CF. Specific inhibitors against the enzyme, in conjunction with current antibiotic treatments and supportive measures, might be a viable near-term approach to improving length and quality of life for many CF patients, before CF gene therapy becomes a reality.

The Penn research team demonstrated the disruptive action of SMase in frog oocytes (egg cells) engineered to place CFTR in their membrane. These oocytes are an experimental tool that allows the researchers to assess the flow of ions across the membrane by measuring electrical current. The researchers found that direct exposure of the CFTR-containing oocytes to SMase of Staphylococcus aureus and Bacillus anthracis bacteria shuts off the electrical current passing through not only the normal, but also the CF-causing mutant CFTR.

The next step for the research team is to develop specific inhibitors against the bacterial SMase and test the idea in an animal model.


'"/>

Source:University of Pennsylvania School of Medicine


Related biology news :

1. Bioartificial kidney under study at MCG
2. W.M. Keck Foundation funds study of friendly microbes
3. Yellowstone microbes fueled by hydrogen, according to U. of Colorado study
4. Genome-wide mouse study yields link to human leukemia
5. Clam embryo study shows pollutant mixture adversely affects nerve cell development
6. New imaging method gives early indication if brain cancer therapy is effective, U-M study shows
7. Same mutation aided evolution in many fish species, Stanford study finds
8. Sequencing of marine bacterium will help study of cell communication
9. Genetically modified rice in China benefits farmers health, study finds
10. A new study examines how shared pathogens affect host populations
11. NYU study reveals how brains immune system fights viral encephalitis
Post Your Comments:
*Name:
*Comment:
*Email:


(Date:3/14/2016)... Florida , March 14, 2016 ... the growing mobile commerce market, announces the airing of a ... channels starting the week of March 21 st .  The ... CNBC, including its popular Squawk on the Street show. ... focused on the growing mobile commerce market, announces the airing ...
(Date:3/11/2016)... --> --> ... Recognition Market by Technology (Pattern Recognition), by Component (Hardware, ... Type (On-Premises and Cloud), by Industry Vertical and by ... the global market is expected to grow from USD ... 2020, at a CAGR of 19.1%. , ...
(Date:3/9/2016)... March 9, 2016 This BCC Research report ... of the RNA Sequencing (RNA Seq) market for the ... instruments, tools and reagents, data analysis, and services. ... of the RNA-Sequencing market such as RNA-Sequencing tools and ... main factors affecting each segment and forecast their market ...
Breaking Biology News(10 mins):
(Date:5/25/2016)... , ... May 25, 2016 , ... Lajollacooks4u has become ... has consistently been rated one of its top attractions. Fortune 500 companies, such ... participate in a unique and intimate team-building experience. , Each event kicks off with ...
(Date:5/25/2016)... ... May 25, 2016 , ... Scientists at the University of Athens say they ... may be hampering the research that could lead to one good one. Surviving Mesothelioma ... it now. , The team evaluated 98 mesothelioma patients who got ...
(Date:5/24/2016)... May 24, 2016   MedyMatch Technology Ltd ., the ... intelligence, real-time decision support tools in the emergency room, announced ... 2016 Israeli Advanced Technology Industries (IATI) BioMed Conference. ... 15th National Life Sciences and Technology Week, and ... Intercontinental Hotel in Tel Aviv, Israel . ...
(Date:5/23/2016)... 23, 2016 Zimmer Biomet Holdings, Inc. (NYSE and ... that its Board of Directors has approved the payment of ... of 2016. The cash dividend of $0.24 ... 2016 to stockholders of record as of the close of ... subject to approval of the Board of Directors and may ...
Breaking Biology Technology: