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Publication : Deficiency in the ALS2 gene does not affect the motor neuron degeneration in SOD1(G93A) transgenic mice.

First Author  Lin X Year  2007
Journal  Neurobiol Aging Volume  28
Issue  10 Pages  1628-30
PubMed ID  16973244 Mgi Jnum  J:125773
Mgi Id  MGI:3759912 Doi  10.1016/j.neurobiolaging.2006.07.014
Citation  Lin X, et al. (2007) Deficiency in the ALS2 gene does not affect the motor neuron degeneration in SOD1(G93A) transgenic mice. Neurobiol Aging 28(10):1628-30
abstractText  Dysfunction of the ALS2 gene has been linked to one form of juvenile onset autosomal recessive amyotrophic lateral sclerosis (ALS). Previous in vitro studies suggest that over-expression of ALS2 protects cells from mutant Cu/Zn superoxide dismutase (SOD1)-induced cytotoxicity. To test whether ALS2 plays a protective role against mutant SOD1-mediated motor neuron degeneration in vivo, we examined the progression of motor neuron disease in SOD1(G93A) mice on an ALS2 null background. Our data suggest that deficiency in the ALS2 gene does not affect the pathogenesis of SOD1(G93A) mice.
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