New insights into functionality of cystic fibrosis protein

September 26, 2012
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 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 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.

Explore further: Gene network restores CF protein function

More information: Jih, K.-Y., et al. 2012. J. Gen. Physiol. 140:347. Tsai, M.-F. 2012. J. Gen. Physiol. doi: 140:343.

Related Stories

Gene network restores CF protein function

August 1, 2012
Researchers at the University of Iowa Carver College of Medicine have discovered a genetic process that can restore function to a defective protein, which is the most common cause of cystic fibrosis (CF).

An 'unconventional' path to correcting cystic fibrosis

September 1, 2011
Researchers have identified an unconventional path that may correct the defect underlying cystic fibrosis, according to a report in the September 2nd issue of the journal Cell. This new treatment dramatically extends the ...

New research could provide roadmap for more effective drug discovery for cystic fibrosis

March 12, 2012
A recent study led by Gergely Lukacs, a professor at McGill University's Faculty of Medicine, Department of Physiology, and published in the January issue of Cell, has shown that restoring normal function to the mutant gene ...

Recommended for you

Want to win at sports? Take a cue from these mighty mice

July 20, 2017
As student athletes hit training fields this summer to gain the competitive edge, a new study shows how the experiences of a tiny mouse can put them on the path to winning.

'Smart' robot technology could give stroke rehab a boost

July 19, 2017
Scientists say they have developed a "smart" robotic harness that might make it easier for people to learn to walk again after a stroke or spinal cord injury.

Engineered liver tissue expands after transplant

July 19, 2017
Many diseases, including cirrhosis and hepatitis, can lead to liver failure. More than 17,000 Americans suffering from these diseases are now waiting for liver transplants, but significantly fewer livers are available.

Lunatic Fringe gene plays key role in the renewable brain

July 19, 2017
The discovery that the brain can generate new cells - about 700 new neurons each day - has triggered investigations to uncover how this process is regulated. Researchers at Baylor College of Medicine and Jan and Dan Duncan ...

New animal models for hepatitis C could pave the way for a vaccine

July 19, 2017
They say that an ounce of prevention is worth a pound of cure. In the case of hepatitis C—a disease that affects nearly 71 million people worldwide, causing cirrhosis and liver cancer if left untreated—it might be worth ...

Omega-3 fatty acids fight inflammation via cannabinoids

July 18, 2017
Chemical compounds called cannabinoids are found in marijuana and also are produced naturally in the body from omega-3 fatty acids. A well-known cannabinoid in marijuana, tetrahydrocannabinol, is responsible for some of its ...

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.