Transgenic Mice Expressing Superoxide Dismutase (SOD)
- Technology Benefits
- Unique in vivo model Widely Used
- Detailed Technology Description
- Development of a unique transgenic mouse model that expresses superoxide dismutase
- *Abstract
-
Northwestern researchers have developed a transgenic mouse model expressing a mutant allele of human superoxide dismutase (SOD) with a Gly93 to Ala93 substitution. The Cu, Zn SOD expressing animals develops clinical features reminiscent of familial amyotrophic lateral sclerosis (ALS). Due to loss of motor neurons in the spinal cord, these hemizygous transgenic mice exhibit rapid motor degeneration and become paralyzed in their limbs and ultimately die within four to five months. This mouse model has already been widely used for screening therapeutic candidates for the discovery of drugs to treat ALS. This is an excellent animal model for the analysis of Cu- or Zn-superoxide dismutase in ALS, also known as Lou Gehrig's disease.
- *Inventors
- Teepu Siddique* Haifeng Pu Eishingdrelo Mark Gurney
- *Publications
- Gurney ME et al., (1994) Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation. Science 264: 1772-5.
- Country/Region
- USA
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