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Dilated cardiomyopathy and neonatal lethality in mutant mice lacking manganese superoxide dismutase.

The Sod2 gene for Mn-superoxide dismutase (MnSOD), an intramitochondrial free radical scavenging enzyme that is the first line of defense against superoxide produced as a byproduct of oxidative phosphorylation, was inactivated by homologous recombination. Homozygous mutant mice die within the first 10 days of life with a dilated cardiomyopathy, accumulation of lipid in liver and skeletal muscle, and metabolic acidosis. Cytochemical analysis revealed a severe reduction in succinate dehydrogenase (complex II) and aconitase (a TCA cycle enzyme) activities in the heart and, to a lesser extent, in other organs. These findings indicate that MnSOD is required for normal biological function of tissues by maintaining the integrity of mitochondrial enzymes susceptible to direct inactivation by superoxide.

Pubmed ID: 7493016


  • Li Y
  • Huang TT
  • Carlson EJ
  • Melov S
  • Ursell PC
  • Olson JL
  • Noble LJ
  • Yoshimura MP
  • Berger C
  • Chan PH
  • Wallace DC
  • Epstein CJ


Nature genetics

Publication Data

December 11, 1995

Associated Grants

  • Agency: NIA NIH HHS, Id: AG08938
  • Agency: NIA NIH HHS, Id: AG10130
  • Agency: NIA NIH HHS, Id: AG13154

Mesh Terms

  • Acidosis
  • Animals
  • Animals, Newborn
  • Base Sequence
  • Cardiomyopathy, Dilated
  • Electron Transport Complex IV
  • Gene Targeting
  • Genes, Lethal
  • Homozygote
  • Lipid Peroxidation
  • Lipids
  • Liver
  • Mice
  • Mice, Mutant Strains
  • Mitochondria, Heart
  • Mitochondria, Muscle
  • Molecular Sequence Data
  • Muscle, Skeletal
  • Sequence Deletion
  • Succinate Dehydrogenase
  • Superoxide Dismutase