Fig. 8

Metabolic and mitochondrial dysfunction were observed following ALS-CSF infusion. (a, b) Tpi-1 activity in mouse spinal cord (a) and brain (b) lysates (n = 5). (c-e) Mitochondrial aggregation. Panel (c) depicts spinal cord sections stained with the mitochondrial marker, mitofilin (green) and NeuN (red). Note the differences in the expression of mitofilin and NeuN within different neurons in the spinal cord from ALS mice (white arrowhead; white, green, and yellow asterisks). Panel (d) represents the patterns of mitochondrial localization within the neurons. (e) denotes the analysis of particle size for the mitofilin staining. (n = 3) Scale Bar = 20 μm. (f-h) Expression patterns and quantification of the immunoblots for ATP5A, from mouse spinal cord (f) (n = 3), and brain (g) (n = 3), as well as human cortical (h) lysates (n = 4, controls; n = 5, ALS). (i.-k.) Expression patterns and quantification of the immunoblots for PGC1α, from mouse spinal cord (i) (n = 3), and brain (j) (n = 3), as well as human cortical(k) lysates (n = 4, controls; n = 5, ALS). (l-n) Expression patterns and quantification of the immunoblots for HSP9A, from mouse spinal cord (l) (n = 3), and brain (m) (n = 3), as well as human cortical(n) lysates. (n = 4, controls; n = 5, ALS). Data are mean ± SEM. (*p ≤ 0.05, **p ≤ 0.01, and *** p ≤ 0.001)