Expression of SOD1* and LC3 in lysosomal and cytoplasmatic fractions of rFFFs and rBM-MSCs from ALS1-PreS and ALS1-EndS animal models. (A,B) Representative Western blotting analysis is expressed as a ratio of the intensity of the SOD1*/SOD1 bands. (C,D) Densitometric analysis is expressed as a ratio of the intensity of the SOD1*/SOD1 bands. Results are reported as the mean± SD of three replicates for each sample. * p < 0.05, ** p < 0.01. (E,F) Representative Western blotting analysis of LC3-I and LC3-II in ALS1 and WT cell fractions. (G,H) Densitometric analysis is expressed as a ratio of the intensity of the LC3-II/LC3-I bands. Results are reported as the mean ± SD of three replicates for each sample. * p < 0.05, ** p < 0.01. (I,J) Activity of Hexosaminidase (HexA + HexB) in ALS1 and WT cell fractions. Enzyme activity was measured by using the specific fluorogenic substrate MUG (see Section 2.14 for details). Results were expressed as the mean ± SD of three independent experiments, each in triplicates. L, lysosomal fraction; C, cytoplasmatic fraction. ns, not significant, * p < 0.05, ** p < 0.01.