Figure 5
From: ALS-linked FUS exerts a gain of toxic function involving aberrant p38 MAPK activation
Increased levels of phosphorylated p38 MAPK in human post-mortem CNS tissues derived from individuals with ALS-FUS. Immunoblot analysis of frozen post-mortem brain (A) and spinal cord (B) tissues derived from non-disease control (C1-C11) and ALS individuals harboring FUS mutations (A1-R521G; A2-H517Q; A3- ex14del; A4-P525L and A5- bp1408del) with the indicated antibodies (see Supplemental Table S2 for detailed patient information). (C) Quantification of phosphorylated (active) p38 (p-p38) by immunoblot analysis revealed higher levels of p-p38 MAPK in ALS cases (cases with levels above the control mean are indicated by red squares), compared to control cases. P-p38 values were normalized to total p38 levels. (D) Quantification of FUS levels in brain normalized to Gapdh. ALS cases with FUS levels above the control mean are indicated by red squares. (C,D) Although, there appears to be a trend in the data, differences between ALS cases and controls did not reach statistical significance due to the small sample size and inherent variability of human postmortem samples.
