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Eggan Lab Creates Mouse Model of Amyotrophic Lateral Sclerosis Using Somatic Cell Nuclear Transfer Cambridge, Mass. (May 2007) – – Kevin Eggan, Ph.D., Assistant Investigator, and his team have created a mouse model of the neurodegenerative disease Amyotrophic Lateral Sclerosis (ALS) — also known as Lou Gehrig’s Disease — using somatic cell nuclear transfer (SCNT). ALS is characterized by the loss of upper and lower motor neurons, culminating in muscle wasting and eventual death from respiratory failure. The advancement was described in “Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model,” published in the May 2007 issue of Nature Neuroscience. The team reported the establishment of an in vitro model system for studying a rare form of ALS that is familial and dominated by mutation of the gene encoding super oxide dismutatase (SOD1). This form of the disease is thought to represent about 2.5 percent of all incidents of ALS. SCNT is often recognized for its potential to treat and cure disease, but it also is expected to play a pivotal role in advancing research through the development of more precise disease models — creating new opportunities to study the origins and progression of disease in the laboratory instead of the clinic or hospital. Additionally, disease models created by SCNT allow researchers to screen drug treatments without administering the drug to patients. “The benefit of this model system is that both the cell types and factors that directly influence motor neuron survival can be investigated,” says Dr. Eggan. “This is an early example of how SCNT can be used to establish valuable model systems for studying complicated diseases.” Dr. Eggan is a member of the Harvard Stem Cell Institute and the Department of Molecular and Cellular Biology at Harvard University. Additional authors include co-equal first authors Franceso Di Giorgio and Monica Carrasco as well as Michelle Siao, and Tom Maniatis. About the Stowers Medical Institute
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