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Waveform rescue and impairment of AIS in C9-MSNs (A) Example AP waveforms for Con-1 (blue) and C9-3 MSNs (black) before and after the addition of BK channel activator NS11021 (10 μM, lighter color shade). Scale bars, 20 mV, 10 ms. (B and C) Mean ± SEM AP amplitude, AHP, half-width, threshold, and rheobase of C9-3 MSNs in the presence and absence of NS11021, along with Con-1 MSNs, were examined in parallel. Statistics, ∗ p < 0.05 and ∗∗∗ p < 0.001, from paired t test or t test . Data: con-1: n = 8, N = 3. C9-3: n = 10, N = 4. (D) NS11021 treatment had no effect on AP output in C9-3 MSNs evoked from current stimulation. Con-1 patched in parallel, shown for reference. Data: Con-1: n = 8, N = 3. C9-3: n = 10, N = 4. Statistics, two-way repeated measures ANOVA followed by Tukey’s multiple comparisons test. (E) Images of Con-1 and C9-3 stained with AIS marker ankyrin-G, neuronal marker <t>MAP2,</t> and DAPI. Scale bars, 50 μm. (F) Mean ± SEM AIS length and distance of start of AIS from the cell soma. Data: Con-1: n = 16, N = 4. C9-3: n = 36, N = 4. ∗∗∗ p < 0.001, t test.
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Waveform rescue and impairment of AIS in C9-MSNs (A) Example AP waveforms for Con-1 (blue) and C9-3 MSNs (black) before and after the addition of BK channel activator NS11021 (10 μM, lighter color shade). Scale bars, 20 mV, 10 ms. (B and C) Mean ± SEM AP amplitude, AHP, half-width, threshold, and rheobase of C9-3 MSNs in the presence and absence of NS11021, along with Con-1 MSNs, were examined in parallel. Statistics, ∗ p < 0.05 and ∗∗∗ p < 0.001, from paired t test or t test . Data: con-1: n = 8, N = 3. C9-3: n = 10, N = 4. (D) NS11021 treatment had no effect on AP output in C9-3 MSNs evoked from current stimulation. Con-1 patched in parallel, shown for reference. Data: Con-1: n = 8, N = 3. C9-3: n = 10, N = 4. Statistics, two-way repeated measures ANOVA followed by Tukey’s multiple comparisons test. (E) Images of Con-1 and C9-3 stained with AIS marker ankyrin-G, neuronal marker <t>MAP2,</t> and DAPI. Scale bars, 50 μm. (F) Mean ± SEM AIS length and distance of start of AIS from the cell soma. Data: Con-1: n = 16, N = 4. C9-3: n = 36, N = 4. ∗∗∗ p < 0.001, t test.
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Waveform rescue and impairment of AIS in C9-MSNs (A) Example AP waveforms for Con-1 (blue) and C9-3 MSNs (black) before and after the addition of BK channel activator NS11021 (10 μM, lighter color shade). Scale bars, 20 mV, 10 ms. (B and C) Mean ± SEM AP amplitude, AHP, half-width, threshold, and rheobase of C9-3 MSNs in the presence and absence of NS11021, along with Con-1 MSNs, were examined in parallel. Statistics, ∗ p < 0.05 and ∗∗∗ p < 0.001, from paired t test or t test . Data: con-1: n = 8, N = 3. C9-3: n = 10, N = 4. (D) NS11021 treatment had no effect on AP output in C9-3 MSNs evoked from current stimulation. Con-1 patched in parallel, shown for reference. Data: Con-1: n = 8, N = 3. C9-3: n = 10, N = 4. Statistics, two-way repeated measures ANOVA followed by Tukey’s multiple comparisons test. (E) Images of Con-1 and C9-3 stained with AIS marker ankyrin-G, neuronal marker <t>MAP2,</t> and DAPI. Scale bars, 50 μm. (F) Mean ± SEM AIS length and distance of start of AIS from the cell soma. Data: Con-1: n = 16, N = 4. C9-3: n = 36, N = 4. ∗∗∗ p < 0.001, t test.
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Waveform rescue and impairment of AIS in C9-MSNs (A) Example AP waveforms for Con-1 (blue) and C9-3 MSNs (black) before and after the addition of BK channel activator NS11021 (10 μM, lighter color shade). Scale bars, 20 mV, 10 ms. (B and C) Mean ± SEM AP amplitude, AHP, half-width, threshold, and rheobase of C9-3 MSNs in the presence and absence of NS11021, along with Con-1 MSNs, were examined in parallel. Statistics, ∗ p < 0.05 and ∗∗∗ p < 0.001, from paired t test or t test . Data: con-1: n = 8, N = 3. C9-3: n = 10, N = 4. (D) NS11021 treatment had no effect on AP output in C9-3 MSNs evoked from current stimulation. Con-1 patched in parallel, shown for reference. Data: Con-1: n = 8, N = 3. C9-3: n = 10, N = 4. Statistics, two-way repeated measures ANOVA followed by Tukey’s multiple comparisons test. (E) Images of Con-1 and C9-3 stained with AIS marker ankyrin-G, neuronal marker <t>MAP2,</t> and DAPI. Scale bars, 50 μm. (F) Mean ± SEM AIS length and distance of start of AIS from the cell soma. Data: Con-1: n = 16, N = 4. C9-3: n = 36, N = 4. ∗∗∗ p < 0.001, t test.
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Waveform rescue and impairment of AIS in C9-MSNs (A) Example AP waveforms for Con-1 (blue) and C9-3 MSNs (black) before and after the addition of BK channel activator NS11021 (10 μM, lighter color shade). Scale bars, 20 mV, 10 ms. (B and C) Mean ± SEM AP amplitude, AHP, half-width, threshold, and rheobase of C9-3 MSNs in the presence and absence of NS11021, along with Con-1 MSNs, were examined in parallel. Statistics, ∗ p < 0.05 and ∗∗∗ p < 0.001, from paired t test or t test . Data: con-1: n = 8, N = 3. C9-3: n = 10, N = 4. (D) NS11021 treatment had no effect on AP output in C9-3 MSNs evoked from current stimulation. Con-1 patched in parallel, shown for reference. Data: Con-1: n = 8, N = 3. C9-3: n = 10, N = 4. Statistics, two-way repeated measures ANOVA followed by Tukey’s multiple comparisons test. (E) Images of Con-1 and C9-3 stained with AIS marker ankyrin-G, neuronal marker <t>MAP2,</t> and DAPI. Scale bars, 50 μm. (F) Mean ± SEM AIS length and distance of start of AIS from the cell soma. Data: Con-1: n = 16, N = 4. C9-3: n = 36, N = 4. ∗∗∗ p < 0.001, t test.
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Waveform rescue and impairment of AIS in C9-MSNs (A) Example AP waveforms for Con-1 (blue) and C9-3 MSNs (black) before and after the addition of BK channel activator NS11021 (10 μM, lighter color shade). Scale bars, 20 mV, 10 ms. (B and C) Mean ± SEM AP amplitude, AHP, half-width, threshold, and rheobase of C9-3 MSNs in the presence and absence of NS11021, along with Con-1 MSNs, were examined in parallel. Statistics, ∗ p < 0.05 and ∗∗∗ p < 0.001, from paired t test or t test . Data: con-1: n = 8, N = 3. C9-3: n = 10, N = 4. (D) NS11021 treatment had no effect on AP output in C9-3 MSNs evoked from current stimulation. Con-1 patched in parallel, shown for reference. Data: Con-1: n = 8, N = 3. C9-3: n = 10, N = 4. Statistics, two-way repeated measures ANOVA followed by Tukey’s multiple comparisons test. (E) Images of Con-1 and C9-3 stained with AIS marker ankyrin-G, neuronal marker <t>MAP2,</t> and DAPI. Scale bars, 50 μm. (F) Mean ± SEM AIS length and distance of start of AIS from the cell soma. Data: Con-1: n = 16, N = 4. C9-3: n = 36, N = 4. ∗∗∗ p < 0.001, t test.
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Waveform rescue and impairment of AIS in C9-MSNs (A) Example AP waveforms for Con-1 (blue) and C9-3 MSNs (black) before and after the addition of BK channel activator NS11021 (10 μM, lighter color shade). Scale bars, 20 mV, 10 ms. (B and C) Mean ± SEM AP amplitude, AHP, half-width, threshold, and rheobase of C9-3 MSNs in the presence and absence of NS11021, along with Con-1 MSNs, were examined in parallel. Statistics, ∗ p < 0.05 and ∗∗∗ p < 0.001, from paired t test or t test . Data: con-1: n = 8, N = 3. C9-3: n = 10, N = 4. (D) NS11021 treatment had no effect on AP output in C9-3 MSNs evoked from current stimulation. Con-1 patched in parallel, shown for reference. Data: Con-1: n = 8, N = 3. C9-3: n = 10, N = 4. Statistics, two-way repeated measures ANOVA followed by Tukey’s multiple comparisons test. (E) Images of Con-1 and C9-3 stained with AIS marker ankyrin-G, neuronal marker <t>MAP2,</t> and DAPI. Scale bars, 50 μm. (F) Mean ± SEM AIS length and distance of start of AIS from the cell soma. Data: Con-1: n = 16, N = 4. C9-3: n = 36, N = 4. ∗∗∗ p < 0.001, t test.
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Waveform rescue and impairment of AIS in C9-MSNs (A) Example AP waveforms for Con-1 (blue) and C9-3 MSNs (black) before and after the addition of BK channel activator NS11021 (10 μM, lighter color shade). Scale bars, 20 mV, 10 ms. (B and C) Mean ± SEM AP amplitude, AHP, half-width, threshold, and rheobase of C9-3 MSNs in the presence and absence of NS11021, along with Con-1 MSNs, were examined in parallel. Statistics, ∗ p < 0.05 and ∗∗∗ p < 0.001, from paired t test or t test . Data: con-1: n = 8, N = 3. C9-3: n = 10, N = 4. (D) NS11021 treatment had no effect on AP output in C9-3 MSNs evoked from current stimulation. Con-1 patched in parallel, shown for reference. Data: Con-1: n = 8, N = 3. C9-3: n = 10, N = 4. Statistics, two-way repeated measures ANOVA followed by Tukey’s multiple comparisons test. (E) Images of Con-1 and C9-3 stained with AIS marker ankyrin-G, neuronal marker <t>MAP2,</t> and DAPI. Scale bars, 50 μm. (F) Mean ± SEM AIS length and distance of start of AIS from the cell soma. Data: Con-1: n = 16, N = 4. C9-3: n = 36, N = 4. ∗∗∗ p < 0.001, t test.
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Waveform rescue and impairment of AIS in C9-MSNs (A) Example AP waveforms for Con-1 (blue) and C9-3 MSNs (black) before and after the addition of BK channel activator NS11021 (10 μM, lighter color shade). Scale bars, 20 mV, 10 ms. (B and C) Mean ± SEM AP amplitude, AHP, half-width, threshold, and rheobase of C9-3 MSNs in the presence and absence of NS11021, along with Con-1 MSNs, were examined in parallel. Statistics, ∗ p < 0.05 and ∗∗∗ p < 0.001, from paired t test or t test . Data: con-1: n = 8, N = 3. C9-3: n = 10, N = 4. (D) NS11021 treatment had no effect on AP output in C9-3 MSNs evoked from current stimulation. Con-1 patched in parallel, shown for reference. Data: Con-1: n = 8, N = 3. C9-3: n = 10, N = 4. Statistics, two-way repeated measures ANOVA followed by Tukey’s multiple comparisons test. (E) Images of Con-1 and C9-3 stained with AIS marker ankyrin-G, neuronal marker <t>MAP2,</t> and DAPI. Scale bars, 50 μm. (F) Mean ± SEM AIS length and distance of start of AIS from the cell soma. Data: Con-1: n = 16, N = 4. C9-3: n = 36, N = 4. ∗∗∗ p < 0.001, t test.
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Image Search Results


Waveform rescue and impairment of AIS in C9-MSNs (A) Example AP waveforms for Con-1 (blue) and C9-3 MSNs (black) before and after the addition of BK channel activator NS11021 (10 μM, lighter color shade). Scale bars, 20 mV, 10 ms. (B and C) Mean ± SEM AP amplitude, AHP, half-width, threshold, and rheobase of C9-3 MSNs in the presence and absence of NS11021, along with Con-1 MSNs, were examined in parallel. Statistics, ∗ p < 0.05 and ∗∗∗ p < 0.001, from paired t test or t test . Data: con-1: n = 8, N = 3. C9-3: n = 10, N = 4. (D) NS11021 treatment had no effect on AP output in C9-3 MSNs evoked from current stimulation. Con-1 patched in parallel, shown for reference. Data: Con-1: n = 8, N = 3. C9-3: n = 10, N = 4. Statistics, two-way repeated measures ANOVA followed by Tukey’s multiple comparisons test. (E) Images of Con-1 and C9-3 stained with AIS marker ankyrin-G, neuronal marker MAP2, and DAPI. Scale bars, 50 μm. (F) Mean ± SEM AIS length and distance of start of AIS from the cell soma. Data: Con-1: n = 16, N = 4. C9-3: n = 36, N = 4. ∗∗∗ p < 0.001, t test.

Journal: Cell Reports

Article Title: Striatal neuron dysfunction in C9ORF72-FTD/ALS is driven by AIS and potassium channel dysregulation

doi: 10.1016/j.celrep.2026.117672

Figure Lengend Snippet: Waveform rescue and impairment of AIS in C9-MSNs (A) Example AP waveforms for Con-1 (blue) and C9-3 MSNs (black) before and after the addition of BK channel activator NS11021 (10 μM, lighter color shade). Scale bars, 20 mV, 10 ms. (B and C) Mean ± SEM AP amplitude, AHP, half-width, threshold, and rheobase of C9-3 MSNs in the presence and absence of NS11021, along with Con-1 MSNs, were examined in parallel. Statistics, ∗ p < 0.05 and ∗∗∗ p < 0.001, from paired t test or t test . Data: con-1: n = 8, N = 3. C9-3: n = 10, N = 4. (D) NS11021 treatment had no effect on AP output in C9-3 MSNs evoked from current stimulation. Con-1 patched in parallel, shown for reference. Data: Con-1: n = 8, N = 3. C9-3: n = 10, N = 4. Statistics, two-way repeated measures ANOVA followed by Tukey’s multiple comparisons test. (E) Images of Con-1 and C9-3 stained with AIS marker ankyrin-G, neuronal marker MAP2, and DAPI. Scale bars, 50 μm. (F) Mean ± SEM AIS length and distance of start of AIS from the cell soma. Data: Con-1: n = 16, N = 4. C9-3: n = 36, N = 4. ∗∗∗ p < 0.001, t test.

Article Snippet: MAP2 anti-Rabbit (1:1000) , Synaptic Systems , Cat# 188 003; RRID AB_2281442.

Techniques: Staining, Marker