Examining the Human Relevance of a New Mouse Model of ALS and Observations on the Incidence in Human Populations Public Deposited

http://ir.library.oregonstate.edu/concern/undergraduate_thesis_or_projects/bc386m15w

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  • Amyotrophic Lateral Sclerosis (ALS) is a relatively rare neurodegenerative disorder that is fatal 2-4 years after the onset of the disease. Population studies are being done in order to help reveal candidates for further studies of disease etiology, disease progression, as well as to further understand racial and ethnic variations. Population-based studies have occurred mostly in the United States and Europe, with few to no studies conducted in other regions. One of the most widely used ALS animal models is the SOD^G93A mouse model. Up until now there has been no effective treatment significantly expanding the lifespan of this model. The Beckman lab has demonstrated that the copper ligand CuATSM effectively halts the progression of ALS symptoms in a variant of the SOD^G93A mouse model co- expressing the Human Copper Chaperone for SOD, hCCS. However, this SOD^G93AxhCCS mouse model is not well-characterized in terms of relative and absolute protein expression levels of SOD compared to CCS. The research done for this thesis aimed to fill this knowledge gap. We demonstrated that the relative expression ratio of SOD1 to hCCS in the SOD^G93AxhCCS mouse model is 1.5:1. These preliminary results will provide insights when comparing this new mouse model to humans.
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