Fig. 4: Skeletal muscle mitochondria of Smn1ΔSkm mice show altered functionality.
a Equal amounts of TA muscle lysates (20 µg) were separated by SDS-PAGE and immunoblotted using the indicated antibodies. Each lane corresponds to an individual mouse of the indicated genotypes. Densitometry analysis yielded the following values: NUDFA9: 0.56 ± 0.05 in Wt (n = 4) and 0.9 ± 0.05 in Smn1ΔSkm (n = 4), p = 0.03; SDHA: 0.28 ± 0.034 in Wt (n = 4) and 0.64 ± 0.036 in Smn1ΔSkm (n = 4), p = 0.03; ATP5F1A: 1.09 ± 0.12 in Wt (n = 4) and 1.57 ± 0.08 in Smn1ΔSkm (n = 4), p = 0.03; TOMM20: 0.5 ± 0.07 in Wt (n = 4) and 1.44 ± 0.26 in Smn1ΔSkm (n = 4), p = 0.03. TUB is used as loading control. b Box-dots plots of respiratory control ratio (RCR) and spare respiratory capacity (SRC) measured in mitochondria isolated from skeletal muscles of Wt and Smn1ΔSkm mice (n = 3). The error bars indicate SEM; *p = 0.045. c BNGE-in-gel activities of MRC CIV from BNGE of digitonin-treated isolated mitochondria from the indicated genotypes. d Mitochondria respiratory chain enzymatic activities normalized by citrate synthase activity in isolated muscle mitochondria from Wt and Smn1ΔSkm muscle (n = 4). e Representative images of gastrocnemius muscle sections from mice of the indicated genotype stained with the NBTx method. Scale bar: 50 µm.
