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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...
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| Autori principali: | , , , , , , , , |
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| Natura: | Artigo |
| Lingua: | Inglês |
| Pubblicazione: |
Oxford University Press
2009
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| Soggetti: | |
| Accesso online: | https://ncbi.nlm.nih.gov/pmc/articles/PMC2644642/ https://ncbi.nlm.nih.gov/pubmed/18818194 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddn309 |
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