Featured Research

from universities, journals, and other organizations

Cystic Fibrosis: Engineered Proteins Can 'Bypass' Genetic Defect

Date:
August 29, 2008
Source:
Federation of American Societies for Experimental Biology
Summary:
By manipulating the machinery used by our cells for quality control, researchers have found a way to restore the function of cystic fibrosis (CF) airway cells. This could significantly reduce the sticky mucus that plugs the lungs of CF patients, which leads to antibiotic-resistant infections and untimely death.

By manipulating the machinery used by our cells for quality control, researchers from the University of Pittsburgh have found a way to restore the function of cystic fibrosis (CF) airway cells. This could significantly reduce the sticky mucus that plugs the lungs of CF patients, which leads to antibiotic-resistant infections and untimely death.

The study is significant because it shows a new way to manipulate the cellular quality controls of all sorts of proteins which play a role in conditions ranging from aging to cancer.

"Our hope is that this work will provide new approaches for recovering function from the protein whose inability to fold and function properly compromises the quality of life and life expectancies of people with CF," said Raymond Frizzell, senior author on the study. The researchers say, however, that it is too early to tell exactly what other conditions this technique will affect.

Most people do not realize that when our bodies produce proteins, they sometimes make mistakes. Similar to what happens on assembly lines, these mistakes are rejected and prevented from being used. In most people with CF, however, defective (i.e., mutant) CFTR proteins made by airway epithelial cells could actually be beneficial if they were allowed to reach their final destination at cell surfaces. To help these mutant proteins (the {DELTA}F508 CF gene mutation) reach cell surfaces, researchers developed a "decoy," made up of only a portion of the complete mutant protein, which is rejected instead. In airway epithelial cells taken from CF patients, the "distracted" airway cells allowed the complete {DELTA}F508 CFTR protein to evade the quality control mechanisms that would have normally destroyed it.

"Since this pivotal discovery of the CF gene nearly 20 years ago, researchers have made more progress toward a cure than they had in thousands of years before," said Gerald Weissmann, MD, Editor-in-Chief of The FASEB Journal. "People with cystic fibrosis now live longer than once ever thought possible, and basic science breakthroughs like this one keep the trend ever upward."

According the U.S. Cystic Fibrosis Foundation, cystic fibrosis affects about 70,000 people worldwide. It is caused by a defective gene and its protein product, which causes the body to produce very thick and sticky mucus. This mucus clogs the lungs and leads to life-threatening lung infections. It also blocks enzymes from the pancreas that needed to break down and absorb food. Sixty years ago, few children with cystic fibrosis lived to attend elementary school. Today, the projected median survival age is 37.


Story Source:

The above story is based on materials provided by Federation of American Societies for Experimental Biology. Note: Materials may be edited for content and length.


Journal Reference:

  1. Sun et al. Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia. The FASEB Journal, 2008; 22 (9): 3255 DOI: 10.1096/fj.07-105338

Cite This Page:

Federation of American Societies for Experimental Biology. "Cystic Fibrosis: Engineered Proteins Can 'Bypass' Genetic Defect." ScienceDaily. ScienceDaily, 29 August 2008. <www.sciencedaily.com/releases/2008/08/080828093347.htm>.
Federation of American Societies for Experimental Biology. (2008, August 29). Cystic Fibrosis: Engineered Proteins Can 'Bypass' Genetic Defect. ScienceDaily. Retrieved July 28, 2014 from www.sciencedaily.com/releases/2008/08/080828093347.htm
Federation of American Societies for Experimental Biology. "Cystic Fibrosis: Engineered Proteins Can 'Bypass' Genetic Defect." ScienceDaily. www.sciencedaily.com/releases/2008/08/080828093347.htm (accessed July 28, 2014).

Share This




More Health & Medicine News

Monday, July 28, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Deadly Ebola Virus Threatens West Africa

Deadly Ebola Virus Threatens West Africa

AP (July 28, 2014) West African nations and international health organizations are working to contain the largest Ebola outbreak in history. It's one of the deadliest diseases known to man, but the CDC says it's unlikely to spread in the U.S. (July 28) Video provided by AP
Powered by NewsLook.com
$15B Deal on Vets' Health Care Reached

$15B Deal on Vets' Health Care Reached

AP (July 28, 2014) A bipartisan deal to improve veterans health care would authorize at least $15 billion in emergency spending to fix a veterans program scandalized by long patient wait times and falsified records. (July 28) Video provided by AP
Powered by NewsLook.com
Two Americans Contract Ebola in Liberia

Two Americans Contract Ebola in Liberia

Reuters - US Online Video (July 28, 2014) Two American aid workers in Liberia test positive for Ebola while working to combat the deadliest outbreak of the virus ever. Linda So reports. Video provided by Reuters
Powered by NewsLook.com
Traditional African Dishes Teach Healthy Eating

Traditional African Dishes Teach Healthy Eating

AP (July 28, 2014) Classes are being offered nationwide to encourage African Americans to learn about cooking fresh foods based on traditional African cuisine. The program is trying to combat obesity, heart disease and other ailments often linked to diet. (July 28) 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:
from the past week

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