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Mouse genetic model for spongiform brain diseases

ITHACA, N.Y. -- Some mice with a genetic mutation for mahogany-colored coats also develop spongiform degeneration of brain tissue, similar to mad cow disease. Because of this oddity, the mice could be valuable animal models for human disorders, such as Parkinson's and Alzheimer's diseases, according to geneticists at Cornell and Stanford universities.

The surprising discovery in a mouse strain known to geneticists since the 1960s is reported in the latest issue of the journal Science (Jan. 31, 2003) by Teresa M. Gunn, Gregory S. Barsh and their collaborators as "Spongiform Degeneration in mahoganoid Mutant Mice."

"Just don't call them mad mice," pleads Gunn, an assistant professor of genetics in Cornell's College of Veterinary Medicine who began the research in Barsh's laboratory at Stanford. "We do see the same kind of tissue degeneration -- with fluid-filled vacuoles, or holes, where the gray matter should be -- in BSE cattle with bovine spongiform encephalopathy and in these mutant mice. But the mice don't have the same motor coordination problems as mad cows, and the condition is not lethal."

Rather, the mutant mice exhibit little more than a slight tremor when they begin to move, they live a normal life span for their species and are able to reproduce, Gunn says. Nor did the investigators find evidence of misshapen prion proteins (the cause of spongiform encephalopathies such as mad cow and mad elk diseases) in the mice, although they did see damage to the myelin sheaths around nerve cells. Among other distinguishing characteristics of the mutant mice are curly whiskers and slightly curly body hair, as well as the habit of clasping their hind feet together when lifted off the ground. Normal mice tend to splay their legs straight out when they are elevated, Gunn explains.

Furthermore, this form of neurodegeneration is not known to be contagious, Gunn says, noting: "A cat that eats a mahoganoid mutant mouse -- shoul
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Contact: Roger Segelken
hrs2@cornell.edu
607-255-9736
Cornell University News Service
30-Jan-2003


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