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CF BUG SPRAY
By Sean Henahan, Access Excellence
BALTIMORE, Md. (June 19, 1997)- An antimicrobial compound
produced by the lungs in response to infection could lead to improved treatments
for cystic fibrosis.
Researchers at Johns Hopkins have identified a compound called hTAP(human
tracheal antimicrobial peptide) that lung cells "squirt" on attacking bacteria.
Significantly, studies of the lungs of patients with lung damage
from cystic fibrosis (CF) show that hTAP is disabled in these patients.
"People have always thought that the lungs only attacked infections
via the classical immune system--B cells, T cells and other immune cells,"
says Pete Pedersen, Ph.D., Hopkins professor of biological chemistry. "But
lung cells apparently have their own first-line defense mechanism--they
shoot out one or more peptides that kill bacteria."
The first such compounds were identified in cells from frog skin and
cow throats by researchers at other institutions Young Hee Ko, a research
associate in Pedersen's lab, suspected that CF might be plugging up the
"nozzle" on a similar bug spray in humans.
"That nozzle is a channel on the surface of lung cells called CFTR,
and we already knew that CF disrupts it," says Ko.
Ko exposed two batches of lung cells, one from a normal patient and
one from a CF patient, to Pseudomonas aeruginosa, a bacterium that causes
repeated lung infections in CF patients. The bacteria infected many
CF cells while making little progress in the normal cells. Ko then searched
for an antimicrobial compound similar to the one found in cow throats,
and found hTAP.
Pedersen speculates that cells in other areas at high risk for infection,
such as the eyes, the gut or the mouth, may secrete similar germ-fighting
compounds. Because hTAP is disabled in CF patients, they have an increased
vulnerability to lung infections. If hTAP or similar compounds can
be mass-produced, they may help fight lung infections in both CF patients
and in the general population, where infectious lung diseases like tuberculosis
kill more than 7 million people every year, said Pedersen.
The Hopkins CF studies were published recently in the journal FEBS (Federation
of European Biochemical Societies) Letters.
CLONED SHEEP MILK
In related research, the British scientists who created Dolly the sheep
clone announced they will begin testing a potential therapy for CF produced
in the milk of genetically engineered sheep.
Twelve volunteers with CF will receive alpha-antitrypsin, a human protein
produced by sheep in their milk. The researchers hope the therapy will
relieve some of the debilitating symptoms of the disease by encouraging
the patients' lungs to repair themselves.
Related information on the Internet
AE: CF Gene
Therapy
AE: Dolly
the Sheep Clone
AE: Antibodies
from Goat Milk
Cystic Fibrosis Foundation
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