Nrf2/ARE Signaling Pathway: Key Mediator in Oxidative Stress and Potential Therapeutic Target in ALS

Table 1

Evidence for protective effects of Nrf2-activation in animal models of neurodegenerative disorders.

Disease	Animal model	Method of Nrf2-activation	Reference
ALS	G93A-SOD1 mice	Synthetic triterpenoids (CDDO-ethylamide (CDDO-EA), CCDO-trifluoroethylamide (CDDO-TFEA))	Neymotin et al., 2011 [29]
Alzheimer’s disease (AD)	Intracerebroventricular infusion of Abeta peptides in rats	Curcumin	Frautschy et al., 2001 [30]
AD	Transgenic APP/PS1 mice	Tert-butylhydroquinone/adenoviral Nrf2 gene transfer	Kanninen et al., 2008 [31]
AD	Transgenic 19959 mice	CDDO-methylamide (CDDO-MA)	Dumont et al., 2009 [32]
AD	Transgenic APP/PS1 mice	Intrahippocampal injection of Nrf2-expressing lentiviral vector	Kanninen et al., 2009 [33]
Parkinson’s disease (PD)	MPTP-toxicity (mice)	Tert-butylhydroquinone	Abdel-Wahab, 2005 [34]
PD	MPTP-toxicity (mice); 3-NP-toxicity (rats)	CDDO-MA	Yang et al., 2009 [35]
PD	MPTP-toxicity (mice)	Astrocytic Nrf2-overexpression	Chen et al., 2009 [36]
PD	Alpha-synuclein expressing Drosophila	Nrf2-overexpression, Keap1-downregulation	Barone et al., 2011 [37]
Huntington’s disease (HD)	Transgenic N171-82Q mice	CDDO-EA, CDDO-TFEA	Stack et al., 2010 [38]
HD	CAG 140 knock in mice	Curcumin	Hickey et al., 2012 [39]