Mouse Model May Lead to Drugs for MSA

Use of this model may advance development of drugs to treat the disease that afflicts some 200,000 Americans and Europeans. A report on this research appears in the June 2002 issue of EMBO Reports (from the European Molecular Biology Organization).


While little is known about the pathology of the disease, it is characterized by the formation of deposits of alpha-synuclein protein into pathologic structures called glial cytoplasmic inclusions in mature brain oligodendrocytes, the cells that form the isolating outer layer surrounding nerve fibers. Normal oligodendrocytes do not produce alpha-synuclein.

Investigators from Ludwig Maximilian University (Munich, Germany) implanted the human gene for alpha-synuclein protein into the genome of a mouse strain. The transgenic mice developed insoluble inclusion bodies of alpha-synuclein in their oligodendrocytes. A significant proportion of the transgenic alpha-synuclein was detergent insoluble, as in MSA patients.

"In patients, the affected cells die as the individual ages. This is something we could not yet observe in our mice, but we are confident that in a next step we can produce mice that will also show this symptom,” said Dr. Philippp Kahle, one of the researchers. "This will help us to understand more about the disease and can help researchers to develop and test drugs against multiple system atrophy.”




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