12. A Model for studying Parkinson’s and Alzheimer’s Diseases

Teresa M. Gunn, Biomedical Sciences, and colleague Gregory S. Barsh (Stanford University) discovered that some mice with a genetic mutation for mahogany-colored coats also develop spongiform degeneration of brain tissue, similar to mad cow disease. Although the same kind of tissue degeneration occurs in BSE cattle with bovine spongiform encephalopathy as in mahoganoid mutant mice, the mice do not experience the same motor coordination problems as mad cows, and the condition is not lethal. The mice have only a slight tremor when they begin to move, they have a normal life span, and they can reproduce. The researchers did not find evidence of deformed prion proteins—the cause of spongiform encephalopathies such as mad cow disease. Because these misshapen prions are not the cause of the mouse condition, the mice are not useful for studying spongiform encephalopathies. However, as an example of defective ubiquitination—a protein-related process involved in Parkinson’s and Alzheimer’s diseases—the mutant mice could become valuable animal models for human neurodegenerative diseases.

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