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UAB's laser-scanning microscope pictures of two
proteins (red and green shapes) involved in Cystic Fibrosis, with
orange tint at the bottom showing interaction.
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Knowing more about this protein proximity will
help scientists better understand the biological underpinnings of CF
and may speed the discovery of new drugs to treat or cure the disease.
CF affects 70,000 people worldwide, 30,000 in the United States.
The findings are published in the Journal of Biological Chemistry,
which included a color photo from the UAB study on its cover.
�Our findings suggest a direct interaction between these two proteins
and their proximity makes the evidence convincing,� said Bakhrom
Berdiev, M.D., Ph.D., a UAB assistant professor of cell biology and
lead author on the study.
The study was conducted using a laser-scanning microscope that
captured images of the two proteins �within reach of each other. The
absence of this interaction could shape the development of CF,�
Berdiev said.
Normally, both proteins help make up the lining of the lungs and other
membranes, and keep the balance of water and salt at safe levels. In
the case of CF, ENaC is allowed to hyper-function in the presence of
the CF-causing protein and disturb the balance of water and salt.
When this happens, a sticky mucus usually forms inside the airways
which can lead to chronic lung infections, the most dangerous symptom
of CF. Other symptoms involve digestive problems.
With the discovery of the CF gene in 1989, many scientists began
searching for ways to repair the DNA or repair the faulty protein
encoded by the DNA.
The new Journal of Biological Chemistry study shows that future
research should look at other molecules not directly linked to CF but
are involved in the disease process and its side effects, Berdiev said.
Also, the new pictures add to an ever-changing biophysical �map� of CF
used to design future research.
The new pictures were done through collaboration between UAB�s
departments of Cell Biology, Physiology & Biophysics, High Resolution
Imaging Facility and the Gregory Fleming James Cystic Fibrosis
Research Center at UAB. Other collaborators are from Ohio State
University, the University of Toronto, Ontario, the National Heart
Lung and Blood Institute in Bethesda, Maryland and Lambert Instruments
in the Netherlands.
Funding for the study came from the National Institutes of Health and
the Cystic Fibrosis Foundation.
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