Maturation and functional restoration of DeltaF508CFTR by its subdomains in cystic fibrosis airway cells

Main Category: Cystic Fibrosis
Article Date: 08 Apr 2005 - 1:00 PDT


Cystic fibrosis is the most common fatal genetic disease among Caucasians, with about 30,000 CF patients in North America.

Researchers tested the notion that introducing a fragment of mutant CFTR (cystic fibrosis transmembrane conductance regulator) would compete for chaperone binding to the full-length mutant and thereby limit its degradation.

Their findings indicate that chaperone competition can rescue delta-F508CFTR from destruction, permitting its channel activity to appear at the cell surface. Also, the delivery of mutant protein fragments may provide a therapy for CF.

Fei Sun, Steven B Condliff, Carol A Bertrand, Zhibao Mi, Joseph M Pilewski, Paul D Robbins, Robert J Bridges, Raymond A Frizzell. Cell Biology and Physiology, University of Pittsburgh. Physiology 349.3. Featured topic 340: "Molecular basis of epithelial disease."

The 35th Congress of the International Union of Physiological Sciences is in San Diego, March 31 - April 5, 2005. The Congress (http://www.iups2005.org) is organized by the six member societies of the U.S. National Committee of the IUPS, the American Physiological Society, the Society for Neuroscience, the Microcirculatory Society, the Society of General Physiologists, the Biomedical Engineering Society, and the Society for Integrative and Comparative Biology, under the auspices of the U.S. National Academy of Sciences.

The IUPS conference, held every four years, runs concurrently this year with Experimental Biology 2005 at the San Diego Convention Center.

Abstracts for all presentations may be accessed through a complete searchable online program at faseb.org/meetings/eb2005/call/default.htm covering both IUPS and Experimental Biology 2005, which is being held concurrently.

The American Physiological Society (APS), which is hosting IUPS, was founded in 1887 to foster basic and applied science, much of it relating to human health. The Bethesda, MD-based Society has more than 10,000 members and publishes nearly 4,000 articles every year in its 14 peer-reviewed journals. In May, APS received the Presidential Award for Excellence in Science, Mathematics and Engineering Mentoring (PAESMEM).

Contact: Mayer Resnick
mresnick@the-aps.org
301-332-4402 (cell)
619-525-6228 (Telephone)
301-634-7209 (office, outside IUPS dates)
The American Physiological Society
http://www.the-aps.org

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