Abstract
Investigation of mitochondrial metabolism perturbations and successful diagnosis of patients with mitochondrial abnormalities often requires assessment of human samples like muscle biopsy. Immunohistochemical and histochemical examination of muscle biopsy is an important technique to investigate mitochondrial dysfunction that combined with spectrophotometric and Blue Native electrophoresis techniques can be an important tool to provide diagnosis of mitochondrial disorders. In this chapter we focus on technical description of the methods that are suitable to detect the activity of complex I, II, and IV of mitochondrial respiratory chain in muscle biopsies. The protocols provided can be useful not only for general assessment of mitochondrial activity in studied material, but they are also successfully used in the diagnostic procedures in case of suspicion of mitochondrial disorders.
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Acknowledgments
This work was supported by the Statutory Funding from Nencki Institute of Experimental Biology, Polish Ministry of Science and Higher Education grant W100/HFSC/2011 and BIO-IMAGing in Research Innovation and Education (FP7-REGPOT-2010-1). Moreover, this work supported by POIG.02.01.00-14-059/09 project titled “Multi-profile modernization of scientific and research infrastructure of The Children’s Memorial Health Institute” that was submitted within the frameworks of the competition for Measure 2.1 “Development of high potential research centres”, Priority II “R&D Infrastructure” within the frameworks of the Innovative Economy Operational Programme, 2007–2013
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Karkucinska-Wieckowska, A., Pronicki, M., Wieckowski, M.R. (2015). Histoenzymatic Methods for Visualization of the Activity of Individual Mitochondrial Respiratory Chain Complexes in the Muscle Biopsies from Patients with Mitochondrial Defects. In: Palmeira, C., Rolo, A. (eds) Mitochondrial Regulation. Methods in Molecular Biology, vol 1241. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-1875-1_8
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DOI: https://doi.org/10.1007/978-1-4939-1875-1_8
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