Scientists observe infectious prion proteins invade and move within brain cells

Scientists for the first time have watched agents of brain-wasting diseases, called transmissible spongiform encephalopathies (TSE), as they invade a nerve cell and then travel along wire-like circuits to points of contact with other cells. These findings will help scientists better understand TSE diseases and may lead to ways to prevent or minimize their effects. TSE, or prion, diseases include scrapie in sheep and goats; chronic wasting disease in deer and elk; mad cow disease in cattle; and Creutzfeldt-Jacob disease in humans.

Under the direction of Byron Caughey, Ph.D., at the Rocky Mountain Laboratories (RML), and Marco Prado, Ph.D., at the University of Minas Gerais in Belo Horizonte, Brazil, the team performed the research in laboratory cultures using a rodent-adapted form of scrapie protein and cells taken from the central nervous system of mouse and hamster brains. The proteins were first "branded" with fluorescent dyes so they could be easily tracked.

The work also revealed that a similar trafficking process might occur with the key plaque-forming protein in Alzheimer's disease, which is not a TSE but a different type of degenerative brain disease, according to Gerald Baron, Ph.D., one of the lead RML researchers. RML, located in Hamilton, MT, is part of the National Institute of Allergy and Infectious Diseases (NIAID) of the National Institutes of Health. The new report appears in the May 25 issue of The Journal of Neuroscience.

"These findings offer intriguing leads toward developing therapies to stop the spread of TSE and possibly other degenerative brain diseases," says NIAID Director Dr. Anthony Fauci. "Potentially, it may be possible to block the pathways that prions use to invade cells, their exit to other cells or their replication within the cells."

Those are precisely some of the next experiments the RML group is pursuing, along with trying to move the fluorescent tracking method from laboratory cell cultures to li

Contact: Ken Pekoc
NIH/National Institute of Allergy and Infectious Diseases

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