Journal: International Journal of Molecular Sciences
Article Title: Nrf2-Inducers Counteract Neurodegeneration in Frataxin-Silenced Motor Neurons: Disclosing New Therapeutic Targets for Friedreich’s Ataxia
doi: 10.3390/ijms18102173
Figure Lengend Snippet: Nrf2-inducers up-regulate the Nrf2 expression in frataxin silenced neurons (shFxn). ( A ) qRT-PCR of mRNA Nrf2 levels in shFxn and Mock after 5 µM (24 h) sulforaphane (SFN) and 30 µM (24 h) dimethyl fumarate (DMF) treatments. Glyceraldehyde 3-phosphate dehydrogenase (GAPDH) is used for normalization and relative quantification of gene expression was performed according to the 2 −ΔΔ C t method; ( B ) Representative Western blot of DMF- and SFN-treated shFxn neurons; ( C ) Densitometry of Nrf2 protein amounts analyzed by Western Blot. Values represent the mean ± SD of three independent experiments (** p < 0.01 and *** p < 0.001, with respect to Mock; # p < 0.05 and ### p < 0.001, with respect to untreated shFxn).
Article Snippet: Nrf2 inducers have been found to be neuroprotective in the MPTP model of PD [ , , ], in fragile X syndrome [ ], in multiple sclerosis [ , ], and a clinical trial with a novel Nrf2 activator (RTA 408) has been started for the treatment of FA (REATA Pharmaceuticals, ClinicalTrials.gov, NCT02255435).
Techniques: Expressing, Quantitative RT-PCR, Quantitative Proteomics, Gene Expression, Western Blot