Featured Research

from universities, journals, and other organizations

Unraveling a new regulator of cystic fibrosis

Date:
September 19, 2011
Source:
American Physiological Society
Summary:
Cystic fibrosis is caused by a genetic defect in a chloride channel called cystic fibrosis transmembrane conductase regulator. Although scientists do not fully understand how or why this defect occurs, researchers have found a promising clue: a protein called ubiquitin ligase Nedd4L.

Cystic fibrosis (CF), a chronic disease that clogs the lungs and leads to life-threatening lung infections, is caused by a genetic defect in a chloride channel called cystic fibrosis transmembrane conductase regulator (CFTR). Although scientists do not fully understand how or why this defect occurs, a team of researchers at The Hospital for Sick Children (SickKids) in Toronto, Ontario, Canada has found a promising clue: a protein called ubiquitin ligase Nedd4L.

Related Articles


In a study led by Daniela Rotin, PhD, senior scientist at SickKids and professor of biochemistry at the University of Toronto, mice specially bred to lack Nedd4L in the lung developed cystic fibrosis-like lung disease. Dr. Rotin, the lead author of a number of recently published studies on this topic, will discuss the team's findings at the 7th International Symposium on Aldosterone and the ENaC/Degenerin Family of Ion Channels conference sponsored by the American Physiological Society.

Previous studies have shown that CF results when a genetic mutation in CFTR interferes with its ability to deliver signals and instructions for cells to execute. In the case of the mutated gene, the lung cells absorb too much salt via a protein known as epithelial sodium channel (ENaC). Airways become dry and hamper the lungs' ability to clear away mucus and filter out foreign matter and bacteria. As a result, the person with CF becomes susceptible to debilitating infection and disease.

Nedd4L and ENaC

Previous CF research has shown that Nedd4L suppresses ENaC. To confirm the link, Dr. Rotin and her team genetically engineered mice to be born without Nedd4L in the lungs. The mice developed lung disease similar to cystic fibrosis, including inflammation and obstructed airways, and died within 3 weeks of birth. When the researchers sampled tissues from deceased mice, they found that there had been increased ENaC activity.

According to Dr. Rotin, the results indicate options for developing treatments for CF. "If you can enhance Nedd4L function or increase the amount of Nedd4L in the lungs, that may be useful in alleviating symptoms of the disease. Another option is to inhibit ENaC."

About the Conference

The 7th International Symposium on Aldosterone and the ENaC/Degenerin Family of Ion Channels explores the relationship between fluid regulation and hypertension, the cardiovascular system and other systems and is sponsored by the American Physiological Society.

Dr. Rotin's presentation is entitled, "Deletion of the Ubiquitin Ligase Nedd4L in Lung Epithelia Causes Cystic Fibrosis-Like Disease."


Story Source:

The above story is based on materials provided by American Physiological Society. Note: Materials may be edited for content and length.


Cite This Page:

American Physiological Society. "Unraveling a new regulator of cystic fibrosis." ScienceDaily. ScienceDaily, 19 September 2011. <www.sciencedaily.com/releases/2011/09/110919113838.htm>.
American Physiological Society. (2011, September 19). Unraveling a new regulator of cystic fibrosis. ScienceDaily. Retrieved March 29, 2015 from www.sciencedaily.com/releases/2011/09/110919113838.htm
American Physiological Society. "Unraveling a new regulator of cystic fibrosis." ScienceDaily. www.sciencedaily.com/releases/2011/09/110919113838.htm (accessed March 29, 2015).

Share This


More From ScienceDaily



More Health & Medicine News

Sunday, March 29, 2015

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

S. Leone in New Anti-Ebola Lockdown

S. Leone in New Anti-Ebola Lockdown

AFP (Mar. 28, 2015) Sierra Leone imposed a three-day nationwide lockdown Friday for the second time in six months in a bid to prevent a resurgence of the deadly Ebola virus. Duration: 01:17 Video provided by AFP
Powered by NewsLook.com
These Popular Antibiotics Can Cause Permanent Nerve Damage

These Popular Antibiotics Can Cause Permanent Nerve Damage

Newsy (Mar. 27, 2015) A popular class of antibiotic can leave patients in severe pain and even result in permanent nerve damage. Video provided by Newsy
Powered by NewsLook.com
WH Plan to Fight Antibiotic-Resistant Germs

WH Plan to Fight Antibiotic-Resistant Germs

AP (Mar. 27, 2015) The White House on Friday announced a five-year plan to fight the threat posed by antibiotic-resistant bacteria amid fears that once-treatable germs could become deadly. (March 27) Video provided by AP
Powered by NewsLook.com
House Ready to Pass Medicare Doc Bill

House Ready to Pass Medicare Doc Bill

AP (Mar. 26, 2015) In rare bipartisan harmony, congressional leaders pushed a $214 billion bill permanently blocking physician Medicare cuts toward House passage Thursday, moving lawmakers closer to resolving a problem that has plagued them for years. (March 26) Video provided by AP
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