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Fig. 2 | Acta Neuropathologica Communications

Fig. 2

From: ALS-linked misfolded SOD1 species have divergent impacts on mitochondria

Fig. 2

B8H10 and AMF7-63 reactive misfolded SOD1 is present in SOD1G93A spinal mitochondrial fractions. a Immunoprecipitation for misfolded SOD1 in isolated SOD1G93A mitochondria with A5C3, B8H10, DSE2 3H1 and AMF7-63. Mouse (mIgG) and rabbit IgG (rbIgG) serve as controls. Input is 10 μg isolated mitochondria. Upper bands and lower bands correspond to human (hSOD1) and rat (rSOD1) SOD1, respectively. Immunoprecipitation with AMF7-63 resulted in a non-specific band (*) just above human SOD1, regardless of misfolded SOD1 status. Experiment shown is representative of three independent trials. b Immunolabeling of isolated spinal cord and liver mitochondria with misfolded SOD1-specific antibody AMF7-63 from symptomatic SOD1G93A rats and controls (aged matched SOD1WT and non-transgenic rats) by flow cytometry. Misfolded SOD1 positive labeling is determined by comparing to isotype control (rabbit IgG, dotplots in first column) of SOD1G93A sample. Percentage of misfolded SOD1+ events is shown for each tissue and genotype in a representative sample. c Quantification of AMF7-63+ events in spinal cord (green circle) or liver (black square) of symptomatic SOD1G93A rats, and age- matched SOD1WT as well as non-transgenic rats. Data are represented as percent of misfolded AMF7-63+ mitochondria, each dot represents one animal, n = 4 animals per genotype per tissue. d Comparison of spinal cord mitochondrial labeling positive for AMF7-63 (green, circle) or B8H10 (blue, square) in pre-symptomatic (10 and 14 weeks) and symptomatic SOD1G93A rats by flow cytometry. Data are represented as percentage of misfolded SOD1+ mitochondria, each dot represents one animal, (mean), n = 4–11 animals. ** P < 0.01. *** P < 0.001

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