Early-onset liver mtDNA depletion and late-onset proteinuric nephropathy in Mpv17 knockout mice

In humans, MPV17 mutations are responsible for severe mitochondrial depletion syndrome, mainly affecting the liver and the nervous system. To gain insight into physiopathology of MPV17-related disease, we investigated an available Mpv17 knockout animal model. We found severe mtDNA depletion in liver...

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Detalles Bibliográficos
Autores principales: Viscomi, Carlo, Spinazzola, Antonella, Maggioni, Marco, Fernandez-Vizarra, Erika, Massa, Valeria, Pagano, Claudio, Vettor, Roberto, Mora, Marina, Zeviani, Massimo
Formato: Texto
Lenguaje:English
Publicado: Oxford University Press 2009
Materias:
Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2644642/
https://www.ncbi.nlm.nih.gov/pubmed/18818194
http://dx.doi.org/10.1093/hmg/ddn309

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2644642/
https://www.ncbi.nlm.nih.gov/pubmed/18818194
http://dx.doi.org/10.1093/hmg/ddn309

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