Navigation Links
Unraveling a new regulator of cystic fibrosis

Bethesda, Md. (Sept. 19, 2011) Cystic fibrosis (CF), a chronic disease that clogs the lungs and leads to life-threatening lung infections, is caused by a genetic defect in a chloride channel called cystic fibrosis transmembrane conductase regulator (CFTR). Although scientists do not fully understand how or why this defect occurs, a team of researchers at The Hospital for Sick Children (SickKids) in Toronto, Ontario, Canada has found a promising clue: a protein called ubiquitin ligase Nedd4L.

In a study led by Daniela Rotin, PhD, senior scientist at SickKids and professor of biochemistry at the University of Toronto, mice specially bred to lack Nedd4L in the lung developed cystic fibrosislike lung disease. Dr. Rotin, the lead author of a number of recently published studies on this topic, will discuss the team's findings at the 7th International Symposium on Aldosterone and the ENaC/Degenerin Family of Ion Channels conference sponsored by the American Physiological Society.

Previous studies have shown that CF results when a genetic mutation in CFTR interferes with its ability to deliver signals and instructions for cells to execute. In the case of the mutated gene, the lung cells absorb too much salt via a protein known as epithelial sodium channel (ENaC). Airways become dry and hamper the lungs' ability to clear away mucus and filter out foreign matter and bacteria. As a result, the person with CF becomes susceptible to debilitating infection and disease.

Nedd4L and ENaC

Previous CF research has shown that Nedd4L suppresses ENaC. To confirm the link, Dr. Rotin and her team genetically engineered mice to be born without Nedd4L in the lungs. The mice developed lung disease similar to cystic fibrosis, including inflammation and obstructed airways, and died within 3 weeks of birth. When the researchers sampled tissues from deceased mice, they found that there had been increased ENaC activity.

According to Dr. Rotin, the results indicate options for developing treatments for CF. "If you can enhance Nedd4L function or increase the amount of Nedd4L in the lungs, that may be useful in alleviating symptoms of the disease. Another option is to inhibit ENaC."


Contact: Donna Krupa
American Physiological Society

Related biology news :

1. Brain structure provides key to unraveling function of bizarre dinosaur crests
2. Physics, math provide clues to unraveling cancer
3. March of Dimes awards $250,000 prize to scientists unraveling the causes of muscular dystrophy
4. New book offers practical advice for unraveling the genetics of complex human diseases
5. Tel Aviv University President Co-authors Important Paper Unraveling the Effect of Spatial Organization on Intracellular Chemistry
6. Unraveling Alzheimers: Simple small molecules could untangle complex disease
7. New regulatory mechanism discovered for cell identify and behavior in forming organs
8. Apolipoprotein(a): A natural regulator of inflammation
9. Jefferson scientists discover a key protein regulator of inflammation and cell death
10. Caltech researchers help unlock the secrets of gene regulatory networks
11. New data suggest jumping genes play a significant role in gene regulatory networks
Post Your Comments:
(Date:11/18/2015)... York , November 18, 2015 ... Research has published a new market report titled  Gesture ... Trends, and Forecast, 2015 - 2021. According to the report, ... 2014 and is anticipated to reach US$29.1 bn by ... 2021. North America dominated ...
(Date:11/17/2015)... , Nov. 17, 2015 Pressure BioSciences, ... in the development and sale of broadly enabling, pressure ... life sciences industry, today announced it has received gross ... $5 million Private Placement (the "Offering"), increasing the total ...  One or more additional closings are expected in the ...
(Date:11/12/2015)... --  Growing need for low-cost, easy to use, ... the way for use of biochemical sensors for ... clinical, agricultural, environmental, food and defense applications. Presently, ... applications, however, their adoption is increasing in agricultural, ... on improving product quality and growing need to ...
Breaking Biology News(10 mins):
(Date:11/30/2015)... , Nov. 30, 2015  HUYA Bioscience International, the ... pharmaceutical innovations, today announced it has signed ... Fund (KDDF) to foster collaboration between KDDF and HUYA ... commercialization of healthcare products for the global market. ... important source of new innovative preclinical and clinical stage ...
(Date:11/30/2015)... Florida and MAGDEBURG, Germany , ... of NeuroRehabilitation (ECNR) in Vienna, Austria ... 3rd European Congress of NeuroRehabilitation (ECNR) in ... --> NovaVision, a wholly owned subsidiary of Vycor ... European version of its Internet-delivered NovaVision Therapy Suite at the ...
(Date:11/30/2015)... Israel , Nov. 30, 2015 BrainStorm ... developer of adult stem cell technologies for neurodegenerative diseases, today ... been awarded an additional grant of approximately $735,000 from ... This grant, the second this year, brings the total awarded ... million (approximately NIS7 million).  ...
(Date:11/27/2015)... ... November 27, 2015 , ... Pittcon is pleased to ... presentations offered in symposia, oral sessions, workshops, awards, and posters. The core ... of applications such as, but not limited to, biotechnology, biomedical, drug discovery, environmental, ...
Breaking Biology Technology: