Lethal mitochondrial cardiomyopathy in a hypomorphic Med30 mouse mutant is ameliorated by ketogenic diet

Deficiencies of subunits of the transcriptional regulatory complex Mediator generally result in embryonic lethality, precluding study of its physiological function. Here we describe a missense mutation in Med30 causing progressive cardiomyopathy in homozygous mice that, although viable during lactat...

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Détails bibliographiques
Auteurs principaux: Krebs, Philippe, Fan, Weiwei, Chen, Yen-Hui, Tobita, Kimimasa, Downes, Michael R., Wood, Malcolm R., Sun, Lei, Li, Xiaohong, Xia, Yu, Ding, Ning, Spaeth, Jason M., Moresco, Eva Marie Y., Boyer, Thomas G., Lo, Cecilia Wen Ya, Yen, Jeffrey, Evans, Ronald M., Beutler, Bruce
Format: Artigo
Langue:Inglês
Publié: National Academy of Sciences 2011
Sujets:
Biological Sciences
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC3241770/
https://ncbi.nlm.nih.gov/pubmed/22106289
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1117835108
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