Figure 2
UMNs are distinguished from other neurons in culture, and changes in average axon length and branching/arborization offer quantitative outcome measures to investigate UMN health (a) UMNs retain their neuronal identity (NF-H + and Ctip2 +) and GFP expression in culture, and they can be distinguished from other neurons that are not an UMN (NF-H + , Ctip2 -). UMNs have large pyramidal cell bodies and long axons. (b-c) UMNs can be visualized and assessed individually in both control WT-UeGFP mice (b), and the hSOD1G93A-UeGFP ALS mouse model (c). (d-e) Bar graph representation of average axon length and (d) and percent distribution of UMN based on average axon length in WT-UeGFP (blue) and hSOD1G93A-UeGFP (red) mice. (f) Sholl analysis of healthy and diseased UMNs with 5 µm circular increments. 0–200 µm radius range is enlarged in the inset for clarification. Mean, SEM, and individual data points shown for n = 3 biological replicates. *p < 0.05, unpaired t-test with Welch's correction. Scale bars = 20 μm, n = 3 biological replicates.
