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

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...

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Publicado en:Free Radic Biol Med
Autores principales: Bennett, Brian, Helbling, Daniel, Meng, Hui, Jarzembowski, Jason, Geurts, Aron M., Friederich, Marisa W., Van Hove, Johan L. K., Lawlor, Michael W., Dimmock, David P.
Formato: Artigo
Lenguaje:Inglês
Publicado: 2016
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Article
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC5047058/
https://ncbi.nlm.nih.gov/pubmed/26773591
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.freeradbiomed.2016.01.001
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