Fig. 7: A small molecule activator of SIRT3, but not riluzole or edaravone, reverses metabolic defects in ALS MNs.
From: ALS motor neurons exhibit hallmark metabolic defects that are rescued by SIRT3 activation
a–c Measurements of basal respiration, ATP production, and spare respiration, respectively, in healthy and ALS MNs treated with DMSO (green) and C12 (blue). Of note, C12 treatment rescued ATP production back to healthy levels. d–f Measurements of basal respiration, ATP production, and spare respiration, respectively, in healthy and ALS MNs treated with DMSO (turquoise) and riluzole (purple). Of note, riluzole treatment is not able to revert ALS MNs mitochondrial bioenergetics defects. g–i Measurements of basal respiration, ATP production, and spare respiration, respectively, in healthy and ALS MNs treated with water (dark blue) and edaravone (dark green). Of note, edaravone treatment is not able to revert ALS MNs mitochondrial bioenergetics defects. j Representative images of ISL1+SMI32+ MNs of BJ-iPS, BJ-SOD1L144F, and BJ-TDP43G298S illustrate only C12 treatment promotes healthier neuronal morphologies, showing cell body sizes (outlined in white dotted lines) from MNs at day 28, and at day 31. Scale bars, 50 μm. ***p < 0.001, ns non-significant; two-tailed t-test.
