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

Academic Article
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Overview

authors

  • Krebs, P.
  • Fan, W. W.
  • Chen, Y. H.
  • Tobita, K.
  • Downes, M. R.
  • Wood, Malcolm R.
  • Sun, L.
  • Li, X. H.
  • Xia, Y.
  • Ding, N.
  • Spaeth, J. M.
  • Moresco, E. M. Y.
  • Boyer, T. G.
  • Lo, C. W. Y.
  • Yen, J.
  • Evans, R. M.
  • Beutler, Bruce

publication date

  • December 2011

journal

  • Proceedings of the National Academy of Sciences of the United States of America  Journal

abstract

  • 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 lactation, show precipitous lethality 2-3 wk after weaning. Expression profiling reveals pleiotropic changes in transcription of cardiac genes required for oxidative phosphorylation and mitochondrial integrity. Weaning mice to a ketogenic diet extends viability to 8.5 wk. Thus, we establish a mechanistic connection between Mediator and induction of a metabolic program for oxidative phosphorylation and fatty acid oxidation, in which lethal cardiomyopathy is mitigated by dietary intervention.

subject areas

  • Amino Acid Sequence
  • Animals
  • Base Sequence
  • Cardiomyopathies
  • Electrophoresis, Polyacrylamide Gel
  • Female
  • Gene Expression
  • Genes, Lethal
  • Kaplan-Meier Estimate
  • Ketogenic Diet
  • Male
  • Mediator Complex
  • Mice
  • Mice, Inbred C3H
  • Mice, Inbred C57BL
  • Microscopy, Electron
  • Mitochondria, Heart
  • Mitochondrial Myopathies
  • Mutation, Missense
  • Myocardium
  • Protein Subunits
  • Reverse Transcriptase Polymerase Chain Reaction
  • Sequence Homology, Amino Acid
  • Sequence Homology, Nucleic Acid
  • Weaning
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Research

keywords

  • heart
  • metabolism
  • peroxisome proliferator-activated receptor-gamma coactivator-1 alpha
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Identity

PubMed Central ID

  • PMC3241770

International Standard Serial Number (ISSN)

  • 0027-8424

Digital Object Identifier (DOI)

  • 10.1073/pnas.1117835108

PubMed ID

  • 22106289
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Additional Document Info

start page

  • 19678

end page

  • 19682

volume

  • 108

issue

  • 49

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