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. 2014 Oct 22;9(10):e110846. doi: 10.1371/journal.pone.0110846

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© 2014 Dombert et al

This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited.

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(A, B) Representative motor endplates from E18 Smn^+/+; SMN2tg and Smn^−/−; SMN2tg Diaphragm stained against Smn and SynPhys. Acetylcholine receptors (AChR) and postsynaptic nuclei were visualized by ω-BTX and DAPI, respectively (scale bar: 5 µm). In (A) Smn deficiency is visible by highly reduced immunoreactive signals, as highlighted in the white box, whereas in (B) the number of Smn particles per NMJ is decreased in SMA type I motor endplates, as indicated by white arrowheads. (A, B) In SMA type I axon terminals (n = 3, N = 32) mean Smn signal intensity was significantly reduced (0.43±0.09, P = 0.0220, t = 6.629, DF = 2) in comparison to control motor endplates (set as ‘1’, n = 3, N = 43), whereas SynPhys signals (Smn^−/−; SMN2tg 1.15±0.19, P = 0.5221, t = 0.7694, DF = 2) and the size of the presynaptic compartment (Control 49.48±13.94 µm²; Smn^−/−; SMN2tg 36.56±7.464; P = 0.4596, t = 0.8174, DF = 4) were comparable. (C) Representative images from E18 Smn^+/+; SMN2tg and Smn^−/−; SMN2tg spinal cord cross sections immunolabeled with Smn and ChAT. Quantitative analysis revealed a significant decrease in cytosolic Smn immunoreactivity in SMA type I motoneurons in comparison to Smn^+/+; SMN2tg cells (Smn^+/+; SMN2tg set as ‘1’, n = 6, N = 107; Smn^−/−; SMN2tg 0.46±0.05, n = 6, N = 85; P<0.0001, t = 11.23, DF = 5). ChAT signal intensity was not statistically affected (Smn^−/−; SMN2tg 0.83±0.21; P = 0.4638, t = 0.7928, DF = 5). (D) Representative Western Blot with cytosolic and nuclear fractions from E18 control and Smn^−/−; SMN2tg spinal cord extracts. Histone H3 and α tubulin were used as markers for nuclear and cytosolic fractions, respectively, and as standardization proteins for quantitative analysis. In SMA type I spinal cord extracts cytosolic and nuclear Smn were significantly reduced by 64% (0.36±0.08, N = 10, P<0.0001, t = 8.480, DF = 9) and 86% (0.14±0.03, N = 10, P<0.0001, t = 26.39, DF = 9), respectively, in comparison to Smn^+/+; SMN2tg extracts (set as ‘1’, N = 10).