Deficiency in the ALS2 gene does not affect the motor neuron degeneration in SOD1G93A transgenic mice

Lin, Xian; Shim, Hoon; Cai, Huaibin

doi:10.1016/j.neurobiolaging.2006.07.014

« Previous Next »Neurobiology of Aging
Volume 28, Issue 10 , Pages 1628-1630, October 2007

Deficiency in the ALS2 gene does not affect the motor neuron degeneration in SOD1^G93A transgenic mice

Unit of Transgenesis, Laboratory of Neurogenetics, National Institute on Aging, National Institutes of Health, Building 35, Room 1A116, MSC 3707, 35 Convent Drive, Bethesda, MD 20892, United States

Received 15 May 2006; received in revised form 7 July 2006; accepted 17 July 2006. published online 29 September 2006.

Abstract

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.

Keywords: Amyotrophic lateral sclerosis (ALS), ALS2, Alsin, SOD1, SOD1^G93A mice