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. 2024 Feb 8;19(3):366–382. doi: 10.1016/j.stemcr.2024.01.002

Figure 2.

Figure 2

p97 R155H motor neurons recapitulate ALS phenotypes

(A) Representative average Fluo-4 fluorescence values from KOLF2.1 motor neurons after 80 mM KCl stimulation (dotted line). The shaded area represents the 95% confidence interval. N = >50 cells per condition.

(B) Percent of KOLF2.1 motor neurons responsive to KCl stimulation (left). Maximal normalized Fluo-4 fluorescence in motor neurons. N > 150 cells, three independent experiments per condition.

(C) Sample trace from a wild-type (WT) KOLF2.1 motor neuron in response to a series of depolarizing current steps via whole-cell patch clamp (left). Input resistance from WT and homozygous motor neurons (middle). Input-output curves of motor neurons in WT and homozygous motor neurons at day 20 (right).

(D) Representative images of TDP-43 immunofluorescence in KOLF2.1 motor neurons (left panels). Quantification of nuclear TDP-43 fluorescence (right). N = >5,000 cells, four independent experiments. Data points represent individual cells; lines represent means. Scale bar, 10 μm.

(E) Representative immunoblots of TDP-43, autophagy markers (p62, LC3b), ER stress markers (ATF4, BIP), and total ubiquitylated proteins (Ub) in KOLF2.1 motor neurons. N = 3 independent experiments.

(F) Normalized viability of KOLF2.1 motor neurons at day 30. All data expressed as means ± SEM unless otherwise indicated. ∗p < 0.05, ∗∗p < 0.01, ∗∗∗p < 0.001, ∗∗∗∗p < 0.0001. One-way ANOVA with Dunnett’s multiple comparison test (B, D, F). See also Figure S3.