Physics Commons^™

Potentially Diagnostic Electron Paramagnetic Resonance Spectra Elucidate The Underlying Mechanism Of Mitochondrial Dysfunction In The Deoxyguanosine Kinase Deficient Rat Model Of A Genetic Mitochondrial Dna Depletion Syndrome, Brian Bennett, Daniel Helbling, Hui Meng, Jason Jarzembowski, Aron M. Geurts, Marisa W. Friederich, Johan L.K. Van Hove, Michael W. Lawlor, David P. Dimmock

Physics Faculty Research and Publications

A novel rat model for a well-characterized human mitochondrial disease, mitochondrial DNA depletion syndrome with associated deoxyguanosine kinase (DGUOK) deficiency, is described. The rat model recapitulates the pathologic and biochemical signatures of the human disease. The application of electron paramagnetic (spin) resonance (EPR) spectroscopy to the identification and characterization of respiratory chain abnormalities in the mitochondria from freshly frozen tissue of the mitochondrial disease model rat is introduced. EPR is shown to be a sensitive technique for detecting mitochondrial functional abnormalities in situ and, here, is particularly useful in characterizing the redox state changes and oxidative stress that can result …