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doi: 10.4061/2011/420580 101

Importantly, mutant SOD1 has been demonstrated to decrease the expression of antioxidant transcription factor NRF2 and its dependent target genes in an established NSC134 cellular model for SOD1-associated ALS (Sarlette et al., 2008) and proteomic analysis of spinal motor neurons from humans with SOD1-related ALS had revealed that the NRF2-induced gene peroxiredoxin is transcriptionally repressed (Wood-Allum et al., 2006)

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