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. 2018 Apr 18;98(2):306–319.e7. doi: 10.1016/j.neuron.2018.03.010

Figure 4.

Figure 4

Cell-Autonomous AS Kir4.1 Loss Does Not Alter MN Survival in ALS

(A) Schematic of iPSC-derived AS from human SOD1D90A ALS patients and non-ALS controls (patient data are provided in Table S2).

(B) Representative images of iPSC-derived AS from human SOD1D90A ALS patients and non-ALS controls labeled with KIR4.1 and GFAP (scale bar, 40 μm).

(C) KCNJ10 mRNA levels are downregulated in SOD1G90A iPSC-derived AS as compared to controls (n = 2–3 independent cultures, mean ± SEM, Mann-Whitney test).

(D) Western blot of KIR4.1, ALDH1L1, and GFAP on iPSC-derived AS from human SOD1D90A ALS patients and non-ALS controls.

(E) Quantification of KIR4.1, ALDH1L1, and GFAP protein levels from western blot in (D) (n = 3/group, mean ± SEM, Mann-Whitney test).

(F) Breeding scheme used for the loss of function of AS Kir4.1 in SOD1G93A (mSOD1) mutant background.

(G) Representative images of ventral horn lumbar spinal cord at P80 from cre-negative control, mSOD1, and mSOD1; AS-Kir4.1cKO (mSOD1; cKO) animals labeled with MN markers (scale bar, 50 μm).

(H and I) Quantification of ChAT+NeuN+ (H) and MMP-9+NeuN+ (I) MN numbers in cre-negative control, mSOD1, and mSOD1; cKO animals (n = 4–7 mice/group, >100 MN counts/animal, mean ± SEM, Kruskal-Wallis test). ∗p < 0.05, ∗∗p < 0.01.