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Protein is key to fatal disorder and normal cell function

ell surface, apoptosis is inappropriately initiated and proceeds uncontrolled, resulting in death of brain cells and the clinical deterioration of juvenile Batten disease.

"We may be able to devise a way to treat juvenile neuronal ceroid lipofuscinosis [Batten disease] by finding an alternate route for delivery of GalCer to the cell surface," Boustany said.

GalCer also interacts with the AIDS virus and beta amyloid, the protein responsible for Alzheimer's disease, and prionic proteins that cause human Creutzfeldt-Jakob Disease and bovine spongiform encephalopathy (mad cow disease). A protein on HIV's surface binds with GalCer and may be important in the transmission of the disease. The beta amyloid and prionic proteins also enter cells by binding to GalCer.


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Contact: Becky Oskin
Becky.oskin@duke.edu
919-684-4148
Duke University Medical Center
15-Sep-2004


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