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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...
| Autores principales: | , , , , , , , , |
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| Formato: | Texto |
| Lenguaje: | English |
| Publicado: |
Oxford University Press
2009
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| Materias: | |
| 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 |