Fig. 2

In vivo neuroprotection of ALS iPSC secretome in the SOD1^G93A mouse ALS model. a Hanging test of the transgenic mice (ALS iPSC-CM-treated mice n = 12 and dF-CM-treated mice n = 6). The decline in performance was significantly delayed in the mice treated with ALS iPSCs-CM, as compared to the dF-CM (114.8 ± 4.29 days vs 91.0 ± 4.31 days, P < 0.01). Values are mean ± SEM. b Kaplan–Meier curves of the disease onset showing that onset was delayed in the ALS iPSC-CM, as compared to the dF-CM group. Onset: (116.8 ± 3.64 days vs 92.3 ± 3.44 days, P < 0.001). c Kaplan–Meier curves of animal survival showing that the ALS iPSC-CM increased lifespan, as compared to dF-CM (141.5 ± 3.26 days vs 120.3 ± 3.07 days, P < 0.001). d Neurological scores were found to be improved by the ALS iPSC-CM, as compared to dF-CM. e Histological analysis (WT for SOD1 C57.B6 mice, n = 6, and ALS mice, n = 8) at 120 days of age, and at the end stage (morbidity time point). Representative images for Cresyl violet staining (and their quantification, bar graph) in the ventral lumbar spinal cord at 120 days showing that ALS iPSC-CM improved the number and the morphology of motor neurons of ALS mice, as compared to the dF-CM. Arrowheads, shrunken MNs. Scale bar, 200 μm. f Representative images of immunostaining of NMJ in gastrocnemius muscle at 120 days and quantification showing that the percent of colocalized NMJ decreased in the dF-CM group, as compared to the WT mice, whereas this percent was improved in the ALS iPSC-CM group. Arrows, colocalized endplates; arrowheads, mis-localized endplates. NF, Neurofilament, SYN, Synaptophysin, AchR, Acetylcholine receptors. Scale bar, 100 μm. g Mass of tibialis anterior (TA) and gastrocnemius (GA) muscles was also increased in the ALS mice treated with ALS iPSC-CM as compared to dF-CM