Extended Data Fig. 6: Denervation and atrophy of skeletal muscle in neuronal specific COXII deficient rats.
From: Mitochondrial respiratory complex IV deficiency recapitulates amyotrophic lateral sclerosis
a-b, Representative images of gastrocnemius (GA) muscle and tibialis anterior (TA) muscle of 5-week COXIILSL and COXIINeun-Cre rats and quantified the muscle weights are presented on the right. Scale bar, 1 cm. Data are presented as mean ± SEM. The statistical test performed was a two-sided unpaired Student’s t-test. c, Representative cross sections of WGA-stains transverse sections of tibialis anterior (TA) muscle in COXIILSL and COXIINeun-Cre rats. scale bar, 200 μm. All experiments were performed independently three times with similar results. d, Quantification of averages cross-section area (CSA) of tibialis. Data are presented as mean ± SEM. The statistical test performed was multiple unpaired Student’s t-tests. e, The representative image shows the neuromuscular junction (NMJ) in hindlimb skeletal muscles of COXIILSL and COXIINeuN-Cre. Motor endplates were stained with a-bungarotoxin to visualize AchR and SV2 was used to label neuromuscular synapses. Scale bar, 20 μm. All experiments were performed independently three times with similar results. f, Quantification of denervated neuromuscular junction (NMJ) in COXIINeuN-Cre rats. Data are presented as mean ± SEM. The statistical test performed was multiple unpaired Student’s t-tests. g-i, Representative images and quantification of CMAPs evoked by supramaximal stimulation of sciatic nerves and the quantification of MUNEs for COXIILSL and COXIINeuN-Cre. Data are presented as mean ± SEM. The statistical test performed was a two-sided unpaired Student’s t-test.
