Figure 5. CHCHD10S55L mice have abnormal neuronal mitochondria and NMJ defects.
A. ChAT immunostaining of motor neurons in lumbar spinal cord. B. Average number of ChAT+ neurons per lumbar spinal cord section. Bars = 50 μm. n=4, 6 sections per mouse. Error bars indicate SEM. n.s., not significantly different by Student’s t-test. C. Electron micrographs of mitochondria in the soma of lumbar spinal cord motor neurons. Arrows indicate intra-mitochondrial vacuolization in CHCHD10S55L motor neurons. Scale bar = 500 μm. D. Quantification of abnormal spinal cord motor neuron mitochondria (% of total mitochondria). E. Gastrocnemius NMJs labeled with TRITC-conjugated BTX (red) and NF-200 (green). Bars = 20 μm. F. Average numbers of innervated (BTX+ and NF-200+) NMJs in gastrocnemius expressed as a percent of total NMJs imaged. n=4, 10 NMJs per mouse. Error bars indicate SEM. *p<0.05 by Student’s t-test. G. Images for morphological analysis of NMJs labeled by BTX taken with fixed settings, and converted to binary images by applying identical thresholds. H. Average NMJ complexity assessed as the total length of branches in each NMJ. I. Average area of NMJs. In H and I, n=4 mice per group, 10 NMJs per mouse. Error bars indicate SEM. *p<0.05, **p<0.01 by Student’s t-test.