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. 2016 May 1;143(9):1547–1559. doi: 10.1242/dev.129676

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© 2016. Published by The Company of Biologists Ltd

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution and reproduction in any medium provided that the original work is properly attributed.

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Fig. 6. — Mitochondrial content and enzyme activity are reduced in Ca_V1.1^ΔE29/ΔE29 muscles. (A,B) SDH activity was analyzed in sections of wild-type (black) and Ca_V1.1^ΔE29/ΔE29 (red) mice. Staining intensity was measured in each fiber profile and plotted in intensity distribution diagrams. (A) In 7-week-old mice, SDH activity is markedly increased in Ca_V1.1^ΔE29/ΔE29 soleus and EDL muscles, as seen by the right-shifted intensity distribution histogram. (B) In 6-month-old mice, SDH activity is significantly reduced in Ca_V1.1^ΔE29/ΔE29 soleus muscle and in EDL muscles. In EDL also the full width at half maximum (FWHM) was reduced. N=3. (C) Electron micrographs of 4- and 5-month-old wild-type and Ca_V1.1^ΔE29/ΔE29 soleus and EDL muscles. Whereas myofibrils and EC coupling membranes appear normal, dilated and lysed mitochondria are found in Ca_V1.1^ΔE29/ΔE29 muscles. (D) Morphometric analysis demonstrates significantly decreased fractional content (percentage area occupied by intact mitochondria) in both Ca_V1.1^ΔE29/ΔE29 muscle types. Mitochondrial size is decreased in Ca_V1.1^ΔE29/ΔE29 muscles and the fraction of damaged mitochondria increased in Ca_V1.1^ΔE29/ΔE29 soleus muscles. N=30-40 images of two biological replicates per condition. *P<0.05, **P<0.01, ***P<0.001. Mean±s.e.m. See also Fig. S3. Scale bars: 100 µm in A,B; 0.5 µm in C.