Featured Research

from universities, journals, and other organizations

New insights into functionality of cystic fibrosis protein

Date:
September 26, 2012
Source:
Rockefeller University Press
Summary:
CFTR is an important protein that, when mutated, causes the life-threatening genetic disease cystic fibrosis. A new study details how an accidental discovery has provided new understanding about CFTR functionality.

A new JGP study provided evidence about the functionality of CFTR, a protein that plays a critical role in cystic fibrosis. Here, an updated model illustrates the relationship between an opening/closing cycle of the gate and ATP consumption in CFTR’s nucleotide-binding domains.
Credit: Jih, K.-Y., et al. 2012. J. Gen. Physiol. 140:347–359.

CFTR is an important protein that, when mutated, causes the life-threatening genetic disease cystic fibrosis. A study in The Journal of General Physiology (JGP) details how an accidental discovery has provided new understanding about CFTR functionality.

From a scientific standpoint, CFTR is unique in that it is the only known ion channel -- a protein pore that enables the passive diffusion of ions across cell membranes -- in the enormous superfamily of ABC proteins, which normally operate as active transporters. As active transporters, ABC proteins use energy derived from ATP hydrolysis to move substrates across the cell membrane against a concentration gradient. Although CFTR is equipped with the same structural elements as that of its ABC family "brethren," it has been unclear whether the ion channel also functions in the same way.

In the October 2012 issue of JGP, Tzyh-Chang Hwang (University of Missouri-Columbia) and colleagues effectively demonstrate that the mechanism through which CFTR functions is indeed akin to that of the ABC transporters. Specifically, the team used a mutant CFTR channel that exhibits two different open states to determine that ATP hydrolysis underlies the unidirectional cycling of CFTR through its open and closed states. This insight provides new evidence about the functionality of a protein that plays an important role in a very prevalent human disease, and continues to be of great interest to researchers.


Story Source:

The above story is based on materials provided by Rockefeller University Press. Note: Materials may be edited for content and length.


Journal Reference:

  1. K.-Y. Jih, Y. Sohma, T.-C. Hwang. Nonintegral stoichiometry in CFTR gating revealed by a pore-lining mutation. The Journal of General Physiology, 2012; 140 (4): 347 DOI: 10.1085/jgp.201210834

Cite This Page:

Rockefeller University Press. "New insights into functionality of cystic fibrosis protein." ScienceDaily. ScienceDaily, 26 September 2012. <www.sciencedaily.com/releases/2012/09/120926123847.htm>.
Rockefeller University Press. (2012, September 26). New insights into functionality of cystic fibrosis protein. ScienceDaily. Retrieved August 21, 2014 from www.sciencedaily.com/releases/2012/09/120926123847.htm
Rockefeller University Press. "New insights into functionality of cystic fibrosis protein." ScienceDaily. www.sciencedaily.com/releases/2012/09/120926123847.htm (accessed August 21, 2014).

Share This




More Health & Medicine News

Thursday, August 21, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Cadavers, a Teen, and a Medical School Dream

Cadavers, a Teen, and a Medical School Dream

AP (Aug. 21, 2014) — Contains graphic content. He's only 17. But Johntrell Bowles has wanted to be a doctor from a young age, despite the odds against him. He was recently the youngest participant in a cadaver program at the Indiana University NW medical school. (Aug. 21) Video provided by AP
Powered by NewsLook.com
Possible Ebola Patient in Isolation at California Hospital

Possible Ebola Patient in Isolation at California Hospital

Reuters - US Online Video (Aug. 20, 2014) — A patient who may have been exposed to the Ebola virus is in isolation at the Kaiser Permanente South Sacramento Medical Center. Linda So reports. Video provided by Reuters
Powered by NewsLook.com
Reasons Why Teen Birth Rates Are At An All-Time Low

Reasons Why Teen Birth Rates Are At An All-Time Low

Newsy (Aug. 20, 2014) — A CDC report says birth rates among teenagers have been declining for decades, reaching a new low in 2013. We look at several popular explanations. Video provided by Newsy
Powered by NewsLook.com
Common Antibiotic Could Lead To Heart-Related Death

Common Antibiotic Could Lead To Heart-Related Death

Newsy (Aug. 20, 2014) — Danish researchers discovered patients taking clarithromycin have an increased risk of dying from a heart-related issue. 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:
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