Mice deleted for heart-type cytochrome c oxidase subunit 7a1 develop dilated cardiomyopathy

Maik Hüttemann, Scott Klewer, Icksoo Lee, Alena Pecinova, Petr Pecina, Jenney Liu, Michael Lee, Jeffrey W. Doan, Douglas Larson, Elise Slack, Bita Maghsoodi, Robert P. Erickson, Lawrence I. Grossman

Research output: Contribution to journalArticle

24 Scopus citations

Abstract

Subunit 7a of mouse cytochrome c oxidase (Cox) displays a contractile muscle-specific isoform, Cox7a1, that is the major cardiac form. To gain insight into the role of this isoform, we have produced a new knockout mouse line that lacks Cox7a1. We show that homozygous and heterozygous Cox7a1 knockout mice, although viable, have reduced Cox activity and develop a dilated cardiomyopathy at 6. weeks of age. Surprisingly, the cardiomyopathy improves and stabilizes by 6. months of age. Cox7a1 knockout mice incorporate more of the "liver-type" isoform Cox7a2 into the cardiac Cox holoenzyme and, also surprisingly, have higher tissue ATP levels.

Original languageEnglish (US)
Pages (from-to)294-304
Number of pages11
JournalMitochondrion
Volume12
Issue number2
DOIs
StatePublished - Mar 1 2012

Keywords

  • Dilated cardiomyopathy
  • Electron transport chain
  • Gene knockout
  • Mitochondria
  • Oxidative phosphorylation

ASJC Scopus subject areas

  • Molecular Medicine
  • Molecular Biology
  • Cell Biology

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  • Cite this

    Hüttemann, M., Klewer, S., Lee, I., Pecinova, A., Pecina, P., Liu, J., Lee, M., Doan, J. W., Larson, D., Slack, E., Maghsoodi, B., Erickson, R. P., & Grossman, L. I. (2012). Mice deleted for heart-type cytochrome c oxidase subunit 7a1 develop dilated cardiomyopathy. Mitochondrion, 12(2), 294-304. https://doi.org/10.1016/j.mito.2011.11.002