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. 2019 Oct 16;8(10):1261. doi: 10.3390/cells8101261

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© 2019 by the authors.

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).

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S100A4 is overexpressed in ALS (amyotrophic lateral sclerosis) human fibroblasts harboring SOD1 pathogenic variants. (A) Immunoblot analysis of total cell extracts showing increased expression of S100A4 in all patients with SOD1 mutations compared with controls. Anti-GAPDH antibody was used to normalize the samples. (B) Representative images of confocal immunofluorescence analysis with phalloidin (green) and anti-S100A4 (red) in a patient with SOD1 G93D mutation. DAPI was used for nuclei staining. Scale bar: 25 μm. Protein lysates of controls and mutant SOD1 fibroblasts were analyzed by western blot using anti-mTOR (C) or anti-NF-κB (D). Anti-GAPDH antibody was used to normalize the samples. The graphs show signal quantification expressed in arbitrary units (AU), relative to controls. Data are reported as mean ± S.E.M. (n = 3 individuals per group). Statistical significance was calculated by student’s t-test. * p < 0.05.

S100A4 is overexpressed in ALS (amyotrophic lateral sclerosis) human fibroblasts harboring SOD1 pathogenic variants. (A) Immunoblot analysis of total cell extracts showing increased expression of S100A4 in all patients with SOD1 mutations compared with controls. Anti-GAPDH antibody was used to normalize the samples. (B) Representative images of confocal immunofluorescence analysis with phalloidin (green) and anti-S100A4 (red) in a patient with SOD1 G93D mutation. DAPI was used for nuclei staining. Scale bar: 25 μm. Protein lysates of controls and mutant SOD1 fibroblasts were analyzed by western blot using anti-mTOR (C) or anti-NF-κB (D). Anti-GAPDH antibody was used to normalize the samples. The graphs show signal quantification expressed in arbitrary units (AU), relative to controls. Data are reported as mean ± S.E.M. (n = 3 individuals per group). Statistical significance was calculated by student’s t-test. * p < 0.05.