Transgenic Mice Expressing Superoxide Dismutase ( 94011)
Researchers at Northwestern University have created a unique transgenic mouse model that expressed superoxide dismutase (SOD). This is an excellent animal model for the analysis of Cu- or Zn-superoxide dismutase in amyotrophic lateral sclerosis (ALS), also called Lou Gehrig's disease, which is a progressive, fatal neurological disease that results from mutations in the Cu, Zn- superoxide dismutase . ALS affects as many as 20,000 Americans with 5,000 new cases occurring in the United States each year and is usually fatal within five years after diagnosis. There is an urgent need for treatment for ALS and similar other debilitating diseases. These transgenic mice have already been widely used for screening therapeutic candidates for the discovery of drugs to treat ALS. [In order to investigate the role of Cu, Zn SOD in the progression of ALS, wild-type and mutant forms of Cu, Zn SOD were expressed in mice. Mutant Cu, Zn SOD expressing animals developed clinical features reminiscent of familial ALS.
FIELD OF APPLICATION: Use of mouse model to investigate role of SOD in development and in disease. Use of ALS disease model to investigate clinical candidates that may ameliorate or eliminate ALS.
Northwestern is interested to have this mouse model utilized for the development of therapeutics to treat ALS and other disease associated with mutations in Cu, Zn SOD. This mouse model is available for licensing.
Further information about this mouse model can be found in various articles, including:
Gurney ME et al., Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation. Science. 1994 Jun 17;264(5166):1772-5.
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