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Fig. 11 | Acta Neuropathologica Communications

Fig. 11

From: Impaired signaling for neuromuscular synaptic maintenance is a feature of Motor Neuron Disease

Fig. 11

Muscle cells cultured from MND patients fail to grow AChR clusters in response to n-agrin treatment. A Myotubes cultured from individual non-MND donors formed large AChR clusters in response to n-agrin. Histograms compare the frequency of large (> 25 µm2) AChR clusters in cultures treated with (+) and without (−) n-agrin. Six out of seven non-MND donors showed an increase in the frequency of large AChR clusters in response to n-agrin treatment (exception being Con-9, see also Additional file 1: Figure S6). B Myotubes cultured from MND patients responded poorly to n-agrin. Apart from MND-14, none of the MND patient cultures showed any increase in the frequency of large (> 25 µm2) AChR clusters in response to n-agrin treatment. C Frequency distributions compare the sizes of AChR clusters on myotubes with and without n-agrin treatment. Each symbol represents the means ± SD for technical replicates; Additional file 1: Figure S6. (C upper graph) Myotubes cultured from non-MND (control) muscle with and without n-agrin treatment (pooled data from 7 donors). (C lower graph) Myotubes cultured from MND patient muscle with and without n-agrin treatment (pooled data from 9 patients: Additional file 1: Figure S7). D After n-agrin treatment, total area of clustered AChRs was significantly increased in control myotubes (n = 7, blue and white symbols). No significant change in total area of clustered AChRs was found in MND myotubes after n-agrin treatment (n = 9, yellow and white symbols; data presented as a log of plus (+) n-agrin over no (minus, −) n-agrin treatment). All data presented as mean ± SD and analyzed by two-way ANOVA with Bonferroni’s multiple comparison test where **p ≤ 0.01

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