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Publication : AIF translocates to the nucleus in the spinal motor neurons in a mouse model of ALS.

First Author  Oh YK Year  2006
Journal  Neurosci Lett Volume  406
Issue  3 Pages  205-10
PubMed ID  16916579 Mgi Jnum  J:112616
Mgi Id  MGI:3662935 Doi  10.1016/j.neulet.2006.07.044
Citation  Oh YK, et al. (2006) AIF translocates to the nucleus in the spinal motor neurons in a mouse model of ALS. Neurosci Lett 406(3):205-10
abstractText  Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by selective loss of motor neurons in the brain stem and the spinal cords. One of the causes for the familial ALS has been attributed to the mutations in copper-zinc superoxide dismutase (SOD1). Although the toxic function of the mutant enzyme has not been fully understood, the final cell death pathway has been suggested as caspase-dependent. In the present study, we present evidence that the activation of apoptosis inducing factor (AIF) may play a role to induce motor neuron death during ALS pathogenesis. In the spinal cord of SOD1 G93A transgenic mice, expression of AIF was detected in the motor neurons and astrocytes. The level of AIF expression increased as the disease progressed. In the symptomatic SOD1 G93A transgenic mice, AIF released from the mitochondria and translocated into the nucleus in the motor neurons as evidenced by confocal microscopy and biochemical analysis. These results suggest that AIF may play a role to induce motor neuron death in a mouse model of ALS.
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