Fig. 6
- ID
- ZDB-FIG-240904-37
- Publication
- Zelina et al., 2024 - ALS-associated C21ORF2 variant disrupts DNA damage repair, mitochondrial metabolism, neuronal excitability and NEK1 levels in human motor neurons
- Other Figures
- All Figure Page
- Back to All Figure Page
Higher spontaneous activity but lower capacity for further stimulation-driven excitability in C21ORF2-V58L motor neurons. A Representative voltage traces in response to 20 pA or 80 pA depolarizing current injections across the three conditions: Healthy control 1 (C1, black), C21ORF2-V58L patient 1 (P1, red), and isogenic control 1 of P1 (iso1, blue). B Average current-action potential relationships in the three conditions: C1 (black; ncells = 48), P1 cells (red; ncells = 46), iso1 (blue; ncells = 31). Two-Way Repeated Measures ANOVA. Current × Genotype interaction. F(38,2318) = 2.082, p = 0.0001. C Left: Bar graph with mean and SEM for action potential number in response to 20pA across the three conditions. One-Way ANOVA: F(2122) = 5.148, p = 0.0071. Tukey post-hoc tests. C1 versus P1, p = 0.0192. C1 versus Iso1, p = 0.9517; P1 versus Iso1, p = 0.0197. Right: Same but in response to 80 pA. One-Way ANOVA: F(2,122) = 4.926, p = 0.0088. Tukey post-hoc tests. C1 versus P1, p = 0.0068. C1 versus Iso1, p = 0.1712; P1 versus Iso1, p = 0.6045. D Bar graph plotted as means with SEM for rheobase across the three conditions. One-Way ANOVA: F(2,122) = 2.317, p = 0.1029. E Bar graphs with the percentage of spontaneously active cells at 0 pA injection steps across the three conditions. C1: 19/48 cells = 40%; P1: 32/46 cells = 70%; Iso1: 18/31 cells = 58%. Kruskall Wallis test = 8.606, p = 0.0135. Dunn’s post-hoc tests. C1 versus P1, p = 0.0108; C1 versus Iso1, p = 0.3245; P1 versus Iso1, p = 0.9647. *p < 0.05; **p < 0.01 |