Featured Research

from universities, journals, and other organizations

Cystic Fibrosis Treatment: New Study On Lung-infecting Bacterial Enzyme Suggests New Approach

Date:
April 19, 2007
Source:
University of Pennsylvania School of Medicine
Summary:
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 patients.

Researchers at the University of Pennsylvania School of Medicine 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.

Related Articles


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.

The National Institute of General Medical Sciences provided funding for this research.


Story Source:

The above story is based on materials provided by University of Pennsylvania School of Medicine. Note: Materials may be edited for content and length.


Cite This Page:

University of Pennsylvania School of Medicine. "Cystic Fibrosis Treatment: New Study On Lung-infecting Bacterial Enzyme Suggests New Approach." ScienceDaily. ScienceDaily, 19 April 2007. <www.sciencedaily.com/releases/2007/04/070418104305.htm>.
University of Pennsylvania School of Medicine. (2007, April 19). Cystic Fibrosis Treatment: New Study On Lung-infecting Bacterial Enzyme Suggests New Approach. ScienceDaily. Retrieved January 27, 2015 from www.sciencedaily.com/releases/2007/04/070418104305.htm
University of Pennsylvania School of Medicine. "Cystic Fibrosis Treatment: New Study On Lung-infecting Bacterial Enzyme Suggests New Approach." ScienceDaily. www.sciencedaily.com/releases/2007/04/070418104305.htm (accessed January 27, 2015).

Share This


More From ScienceDaily



More Health & Medicine News

Tuesday, January 27, 2015

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Ebola Mistakes Should Serve a Lesson Says WHO

Ebola Mistakes Should Serve a Lesson Says WHO

AFP (Jan. 25, 2015) The World Health Organization&apos;s chief on Sunday admitted the UN agency had been caught napping on Ebola, saying it should serve a lesson to avoid similar mistakes in future. Duration: 00:55 Video provided by AFP
Powered by NewsLook.com
Disneyland Measles Outbreak Spreads To 5 States

Disneyland Measles Outbreak Spreads To 5 States

Newsy (Jan. 24, 2015) Much of the Disneyland measles outbreak is being blamed on the anti-vaccination movement. The CDC encourages just about everyone get immunized. Video provided by Newsy
Powered by NewsLook.com
Growing Measles Outbreak Worries Calif. Parents

Growing Measles Outbreak Worries Calif. Parents

AP (Jan. 23, 2015) Public health officials are rushing to contain a measles outbreak that has sickened 70 people across 6 states and Mexico. The AP&apos;s Raquel Maria Dillon has more. (Jan. 23) Video provided by AP
Powered by NewsLook.com
Smart Wristband to Shock Away Bad Habits

Smart Wristband to Shock Away Bad Habits

Reuters - Innovations Video Online (Jan. 23, 2015) A Boston start-up is developing a wristband they say will help users break bad habits by jolting them with an electric shock. Ben Gruber reports. Video provided by Reuters
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:

Breaking News:

Strange & Offbeat Stories


Health & Medicine

Mind & Brain

Living & Well

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile: iPhone Android Web
Follow: Facebook Twitter Google+
Subscribe: RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins