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FOR IMMEDIATE RELEASE
Contact:
Dr. John Cuppoletti, University of Cincinnati College of Medicine,
(513) 558-3022 or john.cuppoletti@uc.edu
NEW COMPOUNDS TEST WAYS TO CORRECT TRANSPORT OF SALT &
WATER ACROSS LUNG, AIRWAYS OF CYSTIC FIBROSIS PATIENTS
One in every 2,500 children born to Caucasian parents has
cystic fibrosis, the most common genetic disease among white Americans.
Cystic fibrosis is characterized by reduced chloride ion permeability,
defects in how salt and water are transported across the epithelial cell
linings in the airways, pancreas, intestine, sweat glands and other organs.
Since water doesn't transport easily, the respiratory and GI tracts become
clogged with mucus. This thick build-up of mucus deposits in the lungs
leads to increased susceptibility toward pulmonary infections. There is no
cure, although recent years have seen great improvement in maintenance
therapy, including effective treatment of secondary infections and new ways
to remove the destructive build up of mucous in the airways. The real trick,
of course, would be to change how salt and water are transported across the
tissues so as to prevent the clogging with mucous. That is what Dr. John
Cuppoletti, University of Cincinnati, is trying to do. He is trying to
circumvent the genetic defect that causes the problem: a cystic fibrosis
transmembrane regulatory protein (CFTR) known to be a chloride channel. At
Experimental Biology 2001, he reports finding additional chloride channels
in the adult human lung and respiratory tissues and also the discovery (and
patenting by his University) of two compounds shown to activate these
additional channels. Relatively simple medication treatments may be able to
"turn on" these other pathways for chloride ion transport, increasing the
salt and water flux in tissues to levels closer to those of normal tissues,
thus reducing life-threatening complications. One of the compounds
developed by Dr. Cuppoletti is currently approved being tested for safety
and biological effects in the nasal tissues of healthy individuals and
cystic fibrosis patients at Children's Hospital, Cincinnati, under the
direction of Dr. Robert Wilmott. If the compounds are found to increase
chloride transport in the nasal tissues in this pilot study, then the next
step would be full scale clinical trials.
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