Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

The Cellular Intricacies of Cystic Fibrosis

20.09.2011
Technique for observing how proteins work in human tissue from cystic fibrosis patients yields new insights into the cellular processes of disease

When researchers discovered the primary genetic defect that causes cystic fibrosis (CF) back in 1989, they opened up a new realm of research into treatment and a cure for the disease. Since then, scientists have been able to clone the defective gene and study its effects in animals. Now researchers at the University of North Carolina at Chapel Hill have developed a technique for observing the defects at work in human tissue donated by patients with CF.

This technique has yielded an extraordinary view of the cellular intricacies of CF, which Martina Gentzsch, assistant professor of cell and developmental biology, will discuss at the 7th International Symposium on Aldosterone and the ENaC/Degenerin Family of Ion Channels, being held September 18-22 in Pacific Grove, Calif. The meeting is sponsored by the American Physiological Society. Her poster presentation is entitled, “The Cystic Fibrosis Transmembrane Conductance Regulator Inhibits Proteolytic Stimulation of ENaC.”

Ion Transport Processes in CF
Cystic fibrosis is caused by a mutation in the gene that encodes a protein called cystic fibrosis transmembrane conductance regulator (CFTR), which functions as a chloride channel at the surface of airways and moves chloride out of the cells. CFTR also regulates another protein called epithelial sodium channel (ENaC), which is responsible for transporting sodium into cells. Thus far, scientists have been able to establish that when the CFTR mutation is present, ENaC becomes overactive and causes the cells in the lungs to absorb too much sodium. Water follows the sodium from the cells’ surfaces into the cells, and as a result, the airways become dry and mucous becomes thick and sticky, leading to infections in the lungs.

To observe how CFTR regulates ENaC, Dr. Gentzsch and her team took cells from healthy lung tissue and CF lung tissue and maintained them in a liquid medium. The cells’ surfaces were exposed to air, which prompted the cells to grow and behave as though they were still inside human lungs. Then the team studied proteolytic cleavage of ENaC, a process in which the ENaC protein is cut by enzymes called proteases at specific sites on the protein. This limited cleavage causes ENaC to become active. When the team analyzed the cells’ behavior, they found that ENaC was more likely to have undergone cleavage in cells from CF tissue.

According to Dr. Gentzsch, these observations prompted two questions. First, what role does CFTR play in regulating ENaC cleavage? Second, why is ENaC cleavage not regulated in CF?

“CFTR binds to ENaC, so our initial thought was that close contact of ENaC to CFTR protects ENaC from being cleaved. But another possibility is that CFTR is responsible for suppressing ENaC cleavage and activation,” said Dr. Gentzsch. In other words, the absence of a normally functioning CFTR protein may cause ENaC overactivity. Because there is more cleavage when the CFTR mutation is present, it implies that healthy CFTR prevents ENaC cleavage and activation, but defective CFTR does not.

Either way, Dr. Gentzsch feels that both CFTR and ENaC should be considered when developing therapies for CF. “Successful treatments should address both decreased CFTR function and increased salt absorption caused by ENaC overactivity.”

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 organ systems. The American Physiological Society is sponsoring the conference which will highlight new scientific findings and offers talks from experts from around the world. Additional information about the conference, being held September 18-22, 2011 in Pacific Grove, CA, can be found online at http://www.the-aps.org/press/releases/11/24.htm.

NOTE TO EDITORS: For a copy of Dr. Gentzsch’s abstract, or to schedule an interview, please contact Donna Krupa at 301.634.7209 or dkrupa@the-aps.org.

Physiology is the study of how molecules, cells, tissues and organs function to create health or disease. The American Physiological Society has been an integral part of the discovery process since it was established in 1887. To keep up with the science, follow @Phyziochick on Twitter.

Donna Krupa | Newswise Science News
Further information:
http://www.the-aps.org

Further reports about: CFTR Cellular ENaC Ionen Pacific coral aldosterone cystic cystic fibrosis fibrosis intricacies lung tissue

More articles from Life Sciences:

nachricht The world's tiniest first responders
21.06.2018 | University of Southern California

nachricht A new toxin in Cholera bacteria discovered by scientists in Umeå
21.06.2018 | Schwedischer Forschungsrat - The Swedish Research Council

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

Die letzten 5 Focus-News des innovations-reports im Überblick:

Im Focus: Temperature-controlled fiber-optic light source with liquid core

In a recent publication in the renowned journal Optica, scientists of Leibniz-Institute of Photonic Technology (Leibniz IPHT) in Jena showed that they can accurately control the optical properties of liquid-core fiber lasers and therefore their spectral band width by temperature and pressure tuning.

Already last year, the researchers provided experimental proof of a new dynamic of hybrid solitons– temporally and spectrally stationary light waves resulting...

Im Focus: Overdosing on Calcium

Nano crystals impact stem cell fate during bone formation

Scientists from the University of Freiburg and the University of Basel identified a master regulator for bone regeneration. Prasad Shastri, Professor of...

Im Focus: AchemAsia 2019 will take place in Shanghai

Moving into its fourth decade, AchemAsia is setting out for new horizons: The International Expo and Innovation Forum for Sustainable Chemical Production will take place from 21-23 May 2019 in Shanghai, China. With an updated event profile, the eleventh edition focusses on topics that are especially relevant for the Chinese process industry, putting a strong emphasis on sustainability and innovation.

Founded in 1989 as a spin-off of ACHEMA to cater to the needs of China’s then developing industry, AchemAsia has since grown into a platform where the latest...

Im Focus: First real-time test of Li-Fi utilization for the industrial Internet of Things

The BMBF-funded OWICELLS project was successfully completed with a final presentation at the BMW plant in Munich. The presentation demonstrated a Li-Fi communication with a mobile robot, while the robot carried out usual production processes (welding, moving and testing parts) in a 5x5m² production cell. The robust, optical wireless transmission is based on spatial diversity; in other words, data is sent and received simultaneously by several LEDs and several photodiodes. The system can transmit data at more than 100 Mbit/s and five milliseconds latency.

Modern production technologies in the automobile industry must become more flexible in order to fulfil individual customer requirements.

Im Focus: Sharp images with flexible fibers

An international team of scientists has discovered a new way to transfer image information through multimodal fibers with almost no distortion - even if the fiber is bent. The results of the study, to which scientist from the Leibniz-Institute of Photonic Technology Jena (Leibniz IPHT) contributed, were published on 6thJune in the highly-cited journal Physical Review Letters.

Endoscopes allow doctors to see into a patient’s body like through a keyhole. Typically, the images are transmitted via a bundle of several hundreds of optical...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

VideoLinks
Industry & Economy
Event News

Munich conference on asteroid detection, tracking and defense

13.06.2018 | Event News

2nd International Baltic Earth Conference in Denmark: “The Baltic Sea region in Transition”

08.06.2018 | Event News

ISEKI_Food 2018: Conference with Holistic View of Food Production

05.06.2018 | Event News

 
Latest News

Better model of water under extreme conditions could aid understanding of Earth's mantle

21.06.2018 | Earth Sciences

What are the effects of coral reef marine protected areas?

21.06.2018 | Life Sciences

The Janus head of the South Asian monsoon

21.06.2018 | Earth Sciences

VideoLinks
Science & Research
Overview of more VideoLinks >>>