Featured Research

from universities, journals, and other organizations

Scientists Discover Basic Defect In Cystic Fibrosis Airway Glands

Date:
March 18, 2006
Source:
American Society for Biochemistry and Molecular Biology
Summary:
Scientists have determined that the buildup of sticky mucus found in cystic fibrosis is caused by a loss in the epithelial cell's ability to secrete fluid. This research appears in the Journal of Biological Chemistry, an American Society for Biochemistry and Molecular Biology journal.

Scientists at Stanford University have determined that the buildup of sticky mucus found in cystic fibrosis is caused by a loss in the epithelial cell's ability to secrete fluid.

Related Articles


Cystic fibrosis is the most common, fatal genetic disease in the United States. It causes the body to produce thick, sticky mucus that builds up in the lungs and blocks the airways. This makes it easy for bacteria to grow and leads to repeated serious lung infections. The thick, sticky mucus can also block tubes in the pancreas, preventing digestive enzymes from reaching the small intestine.

The disorder results from mutations in the gene for the cystic fibrosis transmembrane conductance regulator (CFTR), a membrane channel regulator essential for proper salt and water movement across some epithelia. Currently, there are two essentially opposite explanations for the inability of the body to clear mucus from the airways in cystic fibrosis. The first is that the defective CFTR is unable to aid in fluid secretion in cystic fibrosis airway glands. The second explanation is that the glands still secrete fluid via non-CFTR pathways, but the fluid is reabsorbed by other channels. In fact, it has been proposed that one of CFTR's functions is to inhibit the activity of a channel called the epithelial Na+ channel (ENaC).

Nam Soo Joo and colleagues at Stanford University attempted to determine which hypothesis was correct by measuring the secretion from glands from patients with cystic fibrosis and from normal pigs. They added ENaC inhibitors to the glands to determine if the channel plays a role in mucus clearance. The researchers found no evidence that the inhibitors altered secretion rates in either normal or cystic fibrosis glands. This suggested that loss of CFTR-mediated fluid secretion is the culprit in cystic fibrosis.

"We previously showed that cystic fibrosis airway glands have defective gland secretion in response to certain drugs," explains Joo. "The results of our present study provide evidence that the defective cystic fibrosis gland secretion is not due to a potentially excessive fluid reabsorption pathway within glands but is due to most likely to a lack of fluid secretion from cystic fibrosis glands."

This research appears as the "Paper of the Week" in the March 17 issue of the Journal of Biological Chemistry, an American Society for Biochemistry and Molecular Biology journal.


Story Source:

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


Cite This Page:

American Society for Biochemistry and Molecular Biology. "Scientists Discover Basic Defect In Cystic Fibrosis Airway Glands." ScienceDaily. ScienceDaily, 18 March 2006. <www.sciencedaily.com/releases/2006/03/060318232035.htm>.
American Society for Biochemistry and Molecular Biology. (2006, March 18). Scientists Discover Basic Defect In Cystic Fibrosis Airway Glands. ScienceDaily. Retrieved November 27, 2014 from www.sciencedaily.com/releases/2006/03/060318232035.htm
American Society for Biochemistry and Molecular Biology. "Scientists Discover Basic Defect In Cystic Fibrosis Airway Glands." ScienceDaily. www.sciencedaily.com/releases/2006/03/060318232035.htm (accessed November 27, 2014).

Share This


More From ScienceDaily



More Health & Medicine News

Thursday, November 27, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Pet Dogs to Be Used in Anti-Ageing Trial

Pet Dogs to Be Used in Anti-Ageing Trial

Reuters - Innovations Video Online (Nov. 26, 2014) Researchers in the United States are preparing to discover whether a drug commonly used in human organ transplants can extend the lifespan and health quality of pet dogs. Video provided by Reuters
Powered by NewsLook.com
Today's Prostheses Are More Capable Than Ever

Today's Prostheses Are More Capable Than Ever

Newsy (Nov. 26, 2014) Advances in prosthetics are making replacement body parts stronger and more lifelike than they’ve ever been. Video provided by Newsy
Powered by NewsLook.com
From Popcorn To Vending Snacks: FDA Ups Calorie Count Rules

From Popcorn To Vending Snacks: FDA Ups Calorie Count Rules

Newsy (Nov. 25, 2014) The US FDA is announcing new calorie rules on Tuesday that will require everywhere from theaters to vending machines to include calorie counts. Video provided by Newsy
Powered by NewsLook.com
Daily Serving Of Yogurt Could Reduce Risk Of Type 2 Diabetes

Daily Serving Of Yogurt Could Reduce Risk Of Type 2 Diabetes

Newsy (Nov. 25, 2014) Need another reason to eat yogurt every day? Researchers now say it could reduce a person's risk of developing type 2 diabetes. Video provided by Newsy
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