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
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- 作者：Brian Bennetta ; 1 ; brian.bennett@mu.edu" class="auth_mail" title="E-mail the corresponding author ; Daniel Helblingb ; dhelbling@mcw.edu" class="auth_mail" title="E-mail the corresponding author ; Hui Mengc ; hmeng@mcw.edu" class="auth_mail" title="E-mail the corresponding author ; Jason Jarzembowskic ; jjarzemb@mcw.edu" class="auth_mail" title="E-mail the corresponding author ; Aron M. Geurtsd ; ageurts@mcw.edu" class="auth_mail" title="E-mail the corresponding author ; Marisa W. Friederiche ; Marisa.Friederich@ucdenver.edu" class="auth_mail" title="E-mail the corresponding author ; Johan L.K. Van Hovee ; johan.vanhove@childrenscolorado.org" class="auth_mail" title="E-mail the corresponding author ; Michael W. Lawlorc ; mlawlor@mcw.edu" class="auth_mail" title="E-mail the corresponding author ; David P. Dimmockb ; ddimmock@mcw.edu" class="auth_mail" title="E-mail the corresponding author
- 关键词：COX ; cytochrome oxidase ; M2 ; deoxyguanosine kinase-deficient rat model ; DGUOK ; deoxyguanosine kinase ; EPR (ESR) ; electron paramagnetic (spin) resonance (spectroscopy) ; FeS ; iron&ndash ; sulfur (cluster) ; H&E ; hematoxylin and eosin ; MD ; mitochondrial disease ; MDS ; mitochondrial DNA depletion syndrome ; MPV17 ; mitochondrial inner membrane protein ; mtDNA ; mitochondrial DNA ; NADH ; reduced nicotinamide adenine dinucleotide ; POLG ; DNA polymerase γ ; SDH ; succinate dehydrogenase ; TWINKLE ; a mitochondrial D
- 刊名：Free Radical Biology and Medicine
- 出版年：2016
- 出版时间：March 2016
- 年：2016
- 卷：92
- 期：Complete
- 页码：141-151
- 全文大小：4032 K
文摘
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A rat model of DGUOK deficiency approximates the human disease.
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EPR is sensitive for detecting mitochondrial functional abnormality in situ.
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EPR shows how the respiratory chain is affected by mitochondrial disease.
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EPR has a role in mechanistic pathophysiological studies of mitochondrial disease.
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There is strong potential for EPR as an additional diagnostic tool.