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. Author manuscript; available in PMC: 2019 Sep 1.
Published in final edited form as: Acta Neuropathol. 2018 May 3;136(3):425–443. doi: 10.1007/s00401-018-1852-9

Figure 2. SETX ALS4 mice display motor neuron degeneration and neuron toxicity.

Figure 2

(a) We measured motor neuron area at the L5 level of the lumbar spinal cord in sections from 13 month-old mice of the indicated genotype (n = 3 mice/genotype; 100–120 motor neurons/genotype). **P < .01, ***P < .001; ANOVA with post-hoc Bonferroni test.

(b) We quantified axon numbers, measuring diameters, in the L5 motor root for mice of the indicated genotype (n = 3 mice/genotype; 800 – 850 axons counted/individual mouse).

(c) Comparison of number of large caliber (> 9 μm diameter) motor axons from (b). *P < .05; ANOVA with post-hoc Bonferroni test. Error bars = s.e.m.

(d) Quantification of propidium iodide-positive primary cortical neurons from SETX-L389S+/− mice and littermate control mice (n = 3 mice/genotype; 9 technical replicates/individual mouse). **P < .01, t-test.

(e) We cultured primary cerebellar granule neurons (CGNs) from SETX-L389S+/− mice and littermate control mice in media containing 25 mM KCl, subjected the CGNs to potassium withdrawal by switching them to 5 mM KCl for either 6 hrs or 8 hrs, and then performed immunoblot analysis of CGN protein lysates for cleaved caspase-3. Beta-actin served as the loading control.

(f) Quantification of cleaved caspase-3 level normalized to beta-actin for CGNs subjected to potassium withdrawal for 8 hrs (n = 9 mice/genotype). *P < .05, t-test.

Error bars = s.e.m.