Western blot and MTT datasets supporting NRF2 activation by omaveloxolone in SMA type I human fibroblasts

This dataset contains Western blot quantification, uncropped blots, statistical analyses, and MTT cell viability data generated from control and spinal muscular atrophy (SMA) type I human fibroblasts treated with NRF2-modulating compounds.

The dataset supports the manuscript:"Pharmacological Activation of NRF2 by Omaveloxolone Upregulates NRF2-Target Proteins in SMA Type I Human Fibroblasts"

SMA is caused by loss of SMN protein and is associated with redox imbalance and impaired NRF2 signaling. This dataset investigates whether NRF2 pathway output is altered in SMA fibroblasts and whether it can be pharmacologically modulated.

Primary findings supported by this dataset include:- Reduced basal expression of NRF2 target proteins (NQO1, xCT) and PGC1α in SMA fibroblasts compared to controls- Increased cell viability upon treatment with NRF2 activators, particularly omaveloxolone (OMAV)- Induction of NRF2 target proteins following OMAV treatment in both control and SMA fibroblasts- Modest increase in SMN protein levels in SMA fibroblasts following OMAV treatment

Dataset contents:- Western blot quantification tables (normalized to total protein)- Fold-change calculations relative to untreated and control conditions- GraphPad Prism statistical analysis outputs (ANOVA with Sidak’s multiple comparisons)- MTT assay datasets across multiple time points (24 h, 48 h, 72 h, 96 h)- Raw and processed absorbance values with normalization to vehicle controls- Uncropped Western blot images corresponding to all quantified data- README files describing data processing, normalization strategy, and statistical analysis

Experimental details:- Human fibroblast cell lines derived from controls and SMA type I patients- Treatments include omaveloxolone (OMAV), sulforaphane (SFN), dimethyl fumarate (DMF), and N-acetylcysteine (NAC)- Western blot analyses performed after 48 h treatment- Cell viability assessed by MTT assay at multiple time points- Data derived from independent biological replicates (multiple fibroblast lines per genotype)

This dataset provides full transparency and reproducibility for all quantitative analyses presented in the associated manuscript.